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Complement and Complement Reactions

  • M. Berger
  • C. H. Hammer
  • F. S. Cole
  • H. R. Colten
  • R. Burger
  • C. Rittner
  • P. M. Schneider
  • M. Loos
  • W. Vogt
  • O. Götze
  • K.-H. Büscher
  • W. Opferkuch
  • I. von Zabern
  • G. M. Hänsch
  • U. Rother
  • E. W. Rauterberg
  • M. P. Dierich
  • A.-B. Laurell

Abstract

Introduction. The nine components1 that comprise the classical pathway constitute a family of proteins whose functions can be divided into three phases: recognition, activation, and attack. Most early work in the field focused on defining the functional activities of the various components, leading to our present concept of their reaction sequence. More recently, as modern biochemical techniques have been used to study these proteins, the physicochemical basis for their activities has begun to be elucidated.

References

  1. 1.
    Austen KF, Becker EL, Borsos T, Lachmann PJ, Lepow IH, Mayer MM, Müller-Eberhard HJ, Nelson RA, Rapp HJ, Rosen FS, Trnka Z (1968) Nomenclature of complement. Bull WHO 39:935–938Google Scholar
  2. 1a.
    Reid KBM, Porter RR (1981) The proteolytic activation system of complement. Ann Rev Biochem 50:433–464PubMedGoogle Scholar
  3. 2.
    Ziccardi RJ (1983) The first component of human complement (C1): activation and control. Springer Semin Immunopathol 6:213–230PubMedGoogle Scholar
  4. 3.
    Cooper NR (1983) Activation and regulation of the first complement component. Fed Proc 42:134–138PubMedGoogle Scholar
  5. 4.
    Ziccardi RJ (1981) Activation of the early components of the classical complement pathway under physiologic conditions J Immunol 126:1769–1773PubMedGoogle Scholar
  6. 5.
    Ziccardi RJ (1983) Nature of the metal ion requirement for assembly and function of the first component of complement. J Biol Chem 258:6187–6192PubMedGoogle Scholar
  7. 6.
    Ziccardi RJ (1984) The role of immune complexes in the activation of the first component of human complement. J Immunol 132:283–288PubMedGoogle Scholar
  8. 7.
    Bartholomew RM, Esser AF (1977) The first complement component: evidence for an equilibrium between C1s free in serum and C1s bound in the Cl complex. J Immunol 119:1916–1922PubMedGoogle Scholar
  9. 8.
    Reid KBM, Porter RR (1976) Subunit composition and structure of subcomponent C1q of the first component of human complement. Biochem J 155:19–23PubMedGoogle Scholar
  10. 9.
    Porter RR, Reid KBM (1978) The biochemistry of complement. Nature 275:699–704PubMedGoogle Scholar
  11. 10.
    Mizouchi T, Yonemasu K, Yamashita K, Kobata A (1978) The asparagine linked sugar chains of subcomponent C1q of the first component of human complement. J Biol Chem 253:7404–7409Google Scholar
  12. 11.
    Reid KBM (1976) Isolation, by partial pepsin digestion, of the three collagen-like regions present in subcomponent C1q of the first component of human complement. Biochem J 155:5–17PubMedGoogle Scholar
  13. 12.
    Sim RB, Porter RR, Reid KBM (1977) The structure and enzymic activities of the C1r and C1s subcomponents of C1, the first component of human serum complement. Biochem J 163:219–227PubMedGoogle Scholar
  14. 13.
    Sim RB (1981) The human complement system serine proteases C1r and C1s and their proenzymes. Methods Enzymol 80:26–42PubMedGoogle Scholar
  15. 14.
    Tschopp J (1982) Kinetics of activation of the first component of complement (C1) by IgG oligomers. Mol Immunol 19:651PubMedGoogle Scholar
  16. 15.
    Kasahara T, Takahashi K (1982) Formation of a conformationally changed C1r, a subcomponent of the first component of human complement, as an intermediate of its autoactivation reaction. FEBS Lett 141:128–135PubMedGoogle Scholar
  17. 16.
    Bauer J, Valet G (1981) Conformational changes of the subunits C1q, C1r, and C1s of human complement component Cl demonstrated by 125I labelling. Biochem Biophys Acta 670:129PubMedGoogle Scholar
  18. 17.
    Villiers CL, Chesne S, Lacroix MB, Arlaud GJ, Colomb MG (1982) Structural features of the first component of human complement, C1, as revealed by surface iodination. Biochem J 203:185–193PubMedGoogle Scholar
  19. 18.
    Ziccardi RJ (1980) Specific Cl dissociation immunoassays as an indicator for classical pathway activation and Cl inactivator function. In: Nakamura RM, Dito WR, Tucker III ES (eds) Immunoassays: clinical laboratory techniques for the 1980’s. Liss, New York, pp 433–442Google Scholar
  20. 19.
    Ziccardi RJ, Cooper NR (1979) Active disassembly of the first complement component, C1 by Cl inactivator. J Immunol 123:788–792PubMedGoogle Scholar
  21. 20.
    Kerr MA (1981) The second component of human complement. Methods Enzymol 80:54–64Google Scholar
  22. 21.
    Nagasawa S, Stroud RM (1977) Cleavage of C2 by C1s into the antigenically distinct fragments C2a and C2b: demonstration of binding of C2b to C4b. Proc Natl Acad Sci USA 74:2998–3001PubMedGoogle Scholar
  23. 22.
    Kerr MA (1979) Limited proteolysis of complement components C2 and factor B: structural analogy and limited sequence homology. Biochem J 183:615–622PubMedGoogle Scholar
  24. 23.
    Medicus RG, Götze O, Müller-Eberhard HJ (1976) The serine protease nature of the C3 and C5 convertases of the classical and alternative complement pathways. Scand J Immunol 5:1049–1055PubMedGoogle Scholar
  25. 24.
    Jersild C, Rubinstein P, Day NK (1976) The HLA system and inherited deficiencies of the complement system. Transplant Rev 32:43–71PubMedGoogle Scholar
  26. 25.
    Meo T, Atkinson JP, Bernoco M, Bernoco O, Ceppelini R (1977) Structural heterogeneity of C2 complement protein and its genetic variants in man: a new polymorphism of the HLA region. Proc Natl Acad Sci USA 74:1672PubMedGoogle Scholar
  27. 26.
    Tack BF, Prahl JW (1976) Third component of human complement: purification from plasma and physicochemical characterization. Biochemistry 15:4513–4521PubMedGoogle Scholar
  28. 27.
    Tack BF, Morris SC, Prahl JW (1979) Third compoment of human complement: structural analysis of the polypeptide chains of C3 and C3b. Biochemistry 18:1497–1503PubMedGoogle Scholar
  29. 28.
    Tack BF, Janatova J, Thomas ML, Harrison RA, Hammer CH (1981) The third, fourth, and fifth components of human complement: isolation and biochemical properties. Methods Enzymol 80:64–101Google Scholar
  30. 29.
    Schreiber RD, Müller-Eberhard HJ (1974) Fourth component of human complement: description of a three polypeptide chain structure. J Exp Med 140:1324–1334PubMedGoogle Scholar
  31. 30.
    Gigli I, von Zabern I, Porter RR (1977) The isolation and structure of C4, the fourth component of human complement. Biochem J 165:439–446PubMedGoogle Scholar
  32. 31.
    Bolotin C, Morris S, Tack B, Prahl J (1977) Purification and structural analysis of the fourth component of human complement. Biochemistry 16:2008–2015PubMedGoogle Scholar
  33. 32.
    Law SK, Levine RP (1977) Interaction between the third complement protein and cell surface macromolecules. Proc Natl Acad Sci USA 74:2701–2705PubMedGoogle Scholar
  34. 33.
    Goers JWF, Porter RR (1978) The assembly of early components of complement on anti-body-antigen aggregates and on antibody-coated erythrocytes. Biochem J 175:675–684PubMedGoogle Scholar
  35. 34.
    Law SK, Lichtenberg NA, Levine RP (1979) Evidence for an ester linkage between the labile binding site of C3b and receptive surfaces. J Immunol 123:1388–1394PubMedGoogle Scholar
  36. 35.
    Law SK, Fearon DT, Levine RP (1979) Action of the C3b-inactivator on cell-bound C3b. J Immunol 122:759–765PubMedGoogle Scholar
  37. 36.
    Law SK, Lichtenberg NA, Levine RP (1980) Covalent binding and hemolytic activity of complement proteins. Proc Natl Acad Sci USA 77:7194–7198PubMedGoogle Scholar
  38. 37.
    Campbell RD, Dodds AW, Porter RR (1980) The binding of human complement component C4 to antibody-antigen aggregates. Biochem J 189:67–80PubMedGoogle Scholar
  39. 38.
    Law SK, Lichtenberg NA, Holcombe FH, Levine RP (1980) Interaction between the labile binding sites of the fourth (C4) and fifth (C5) complement proteins and erythrocyte cell membranes. J Immunol 125:634–639PubMedGoogle Scholar
  40. 39.
    Janatova J, Lorenz PE, Schechter AN, Prahl JW, Tack BF (1980) Third component of human complement: appearance of a sulfhydryl group following chemical or enzymatic inactivation. Biochemistry 19:4471–4478PubMedGoogle Scholar
  41. 40.
    Janatova J, Tack BF, Prahl JW (1980) Third component of human complement: structural requirements for its function. Biochemistry 19:4479–4485PubMedGoogle Scholar
  42. 41.
    Pangburn MK, Müller-Eberhard HJ (1980) Relation of a putative thioester bond in C3 to activation of the alternative pathway and the binding of C3b to biological targets of complement. J Exp Med 152:1102–1114PubMedGoogle Scholar
  43. 42.
    Lundwall A, Malmheden I, Heilman U, Sjöquist (1981) Interaction between the labile binding site of human C4 and methylamine. Scand J Immunol 13:199–203PubMedGoogle Scholar
  44. 43.
    Gorski JP, Silversmith R, Fiebeger S, Moilanen T (1982) Covalent modification of fourth component of human complement with primary amines. J Biol Chem 257:10948–10954PubMedGoogle Scholar
  45. 44.
    Tack BF, Harrison RA, Janatova J, Thomas ML, Prahl JW (1980) Evidence for presence of an internal thiolester bond in third component of human complement. Proc Natl Acad Sci USA 77:5764–5768PubMedGoogle Scholar
  46. 45.
    Thomas ML, Janatova J, Gray WR, Tack BF (1982) Third component of human complement: localization of the internal thiolester bond. Proc Natl Acad Sci USA 79:1054–1058PubMedGoogle Scholar
  47. 46.
    Janatova J, Tack BF (1981) Fourth component of human complement: studies of an amine-sensitive site comprised of a thiol component. Biochemistry 20:2394–2402PubMedGoogle Scholar
  48. 47.
    Harrison RA, Thomas ML, Tack BF (1981) Sequence determination of the thiolester site of the fourth component of human complement. Proc Natl Acad Sci USA 78:7388–7392PubMedGoogle Scholar
  49. 48.
    Hostetter MK, Thomas ML, Rosen FS, Tack BF (1982) Binding of C3b proceeds by a transesterification reaction at the thiolester site. Nature 298:72–75PubMedGoogle Scholar
  50. 49.
    Mann J, O’Brien R, Hostetter MK, Alper CA, Rosen FS, Babior BM (1981) The third component of complement: covalent attachment of a radioactive sugar to the labile binding site of C3 via the alternative pathway. J Immunol 126:2370–2372PubMedGoogle Scholar
  51. 50.
    Capel PJA, Groeneboer O, Grosveld G, Pondman KW (1978) The binding of activated C3 to polysaccharides and immunoglobulins. J Immunol 121:2566–2572PubMedGoogle Scholar
  52. 51.
    Sim RB, Twose TM, Paterson DS, Sim E (1981) The covalent-binding reaction of complement component C3. Biochem J 193:115–127PubMedGoogle Scholar
  53. 52.
    Law SKA, Minich TM, Levine RP (1981) Binding reaction between the third human complement protein and small molecules. Biochemistry 20:7457–7463PubMedGoogle Scholar
  54. 53.
    Law SKA (1983) Non-enzymatic activation of the covalent binding reaction of the complement protein C3, Biochem J 211:381–389PubMedGoogle Scholar
  55. 54.
    Isenman DE, Kells DIC, Cooper NR, Müller-Eberhard HJ, Pangburn MK (1981) Nucleo-philic modification of human complement protein C3: correlation of conformational changes with acquisition of C3b-like functional properties. Biochemistry 20:4458–4467PubMedGoogle Scholar
  56. 55.
    Isenman DE, Cooper NR (1981) The structure and function of the third component of human complement-I. The nature and extent of conformational changes accompanying C3 activation. Mol Immunol 18:331–339PubMedGoogle Scholar
  57. 56.
    Isenman DE, Kells DI (1982) Conformational and functional changes in the fourth component of human complement produced by nucleophilic modification and by proteolysis with C1s. Biochemistry 21:1109–1117PubMedGoogle Scholar
  58. 57.
    Isenman DE (1983) Conformational changes accompanying proteolytic cleavage of human complement protein C3b by the regulatory enzyme factor I and its cofactor H. J Biol Chem 258:4238–4244PubMedGoogle Scholar
  59. 58.
    von Zabern I, Nolte R, Vogt W (1981) Treatment of human complement components C4 and C3 with amines or chaotropic ions. Scand J Immunol 13:413–431Google Scholar
  60. 59.
    von Zabern I, Bloom EL, Chu V, Gigli I (1982) The fourth component of human complement treated with amines or chaotropes or frozen-thawed (C4b-like C4): interaction with C4 binding protein and cleavage by C3b/C4b inactivator. J Immunol 128:1433–1438Google Scholar
  61. 60.
    Pangburn MK, Schreiber RD, Müller-Eberhard HJ (1981) Formation of the initial C3 convertase of the alternative complement pathway. J Exp Med 154:856–867PubMedGoogle Scholar
  62. 61.
    Berger M, Gaither TA, Hammer CH, Frank MM (1981) Lack of binding of human C3, in its native state, to C3b receptors. J Immunol 127:1329–1334PubMedGoogle Scholar
  63. 62.
    Dixit R, Schneider R, Law SK, Kulczycki A Jr, Atkinson JP (1981) Ligand binding specificity of a rabbit alveolar macrophage receptor for C3b. J Biol Chem 257:1595–1597Google Scholar
  64. 63.
    Gorski JP, Howard JB (1980) Effect of methylamine on the structure and function of the fourth component of human complement, C4. J Biol Chem 255:10025–10028PubMedGoogle Scholar
  65. 64.
    Parkes C, DiScipio RG, Kerr MA, Prohaska R (1981) The separation of functionally distinct forms of the third component of human complement (C3). Biochem J 193:963–970PubMedGoogle Scholar
  66. 65.
    Müller-Eberhard HJ, Nilsson U, Aronsson T (1960) Isolation and characterization of two β 1-glycoproteins of human serum. J Exp Med 111:201–215PubMedGoogle Scholar
  67. 66.
    Müller-Eberhard HJ, Nilsson U (1960) Relation of a β 1-glycoprotein of human serum to the complement system. J Exp Med 111:217–234PubMedGoogle Scholar
  68. 67.
    West CD, Davis NC, Forristal J, Herbst J, Spitzer R (1966) Antigenic determinants of human β 1C - and β 1G-globulins. J Immunol 96:650–658PubMedGoogle Scholar
  69. 68.
    Spitzer RE, Stitzel AE, Pauling VL, Davis NC, West CD (1971) The antigenic and molecular alterations of C3 in the fluid phase during an immune reaction in normal human serum. J Exp Med 134:656–680PubMedGoogle Scholar
  70. 69.
    Molenaar JL, ten Velde A, Pondman KW (1973) Anti-C3a, anti-C3b (anti-B) and their reaction with the anaphylatoxic fragment C3a. J Immunol 110:702–708PubMedGoogle Scholar
  71. 70.
    Molenaar JL, Müller MAC, Engelfriet CP, Pondman KW (1974) Changes in antigenic properties of human C3 upon activation and conversion by trypsin. J Immunol 112:1444–1451PubMedGoogle Scholar
  72. 71.
    Asghar SS, Cormane RH (1976) Interaction of the B-determinant of the third component of complement with amidino compounds. Immunochemistry 13:975–978PubMedGoogle Scholar
  73. 72.
    Lachmann PJ, Oldroyd RG, Milstein C, Wright BW (1980) Three rat monoclonal antibodies to human C3. Immunology 41:503–515PubMedGoogle Scholar
  74. 73.
    Lachmann PJ, Pangburn MK, Oldroyd RG (1982) Breakdown of C3 after complement activation. J Exp Med 156:205–216PubMedGoogle Scholar
  75. 74.
    Tack BF, Morris SC, Prahl JW (1979) Fifth component of human complement: purification from plasma and polypeptide chain structure. Biochemistry 18:1490–1497PubMedGoogle Scholar
  76. 75.
    Fernandez HN, Hugli TE (1976) Partial characterization of human C5a anaphylatoxin. J Immunol 117:1688–1694PubMedGoogle Scholar
  77. 76.
    Fernandez HN, Hugli TE (1977) Chemical evidence for common genetic ancestry of complement components C3 and C5. J Biol Chem 252:1826–1828PubMedGoogle Scholar
  78. 77.
    Gerard C, Hugli TE (1981) Identification of classical anaphylatoxin as the des-Arg form of the C5a molecule: evidence of a modulator role for the oligosaccharide unit in human des- Arg-C5a. Proc Natl Acad Sci USA 78:1833–1837PubMedGoogle Scholar
  79. 78.
    Wetsel RA, Kolb WP (1982) Complement-independent activation of the fifth component (C5) of human complement: limited trypsin digestion resulting in the expression of biologic activity. J Immunol 128:2209–2216PubMedGoogle Scholar
  80. 79.
    Wetsel RA, Kolb WP (1983) Expression of C5a-like biological activities by the fifth component of human complement (C5) upon limited digestion with noncomplement enzymes without release of polypeptide fragments. J Exp Med 157:2029–2048PubMedGoogle Scholar
  81. 80.
    Kolb WP, Kolb LM, Savary JR (1982) Biochemical Characterization of the sixth component of complement. Biochemistry 21:294–301PubMedGoogle Scholar
  82. 81.
    Discipio RG, Gagnon J (1982) Characterization of human complement components C6 and C7. Mol Immunol 19:1425–1431PubMedGoogle Scholar
  83. 82.
    Podack ER, Kolb WP, Müller-Eberhard HJ (1976) Purification of the sixth and seventh component of human complement without loss of hemolytic activity. J Immunol 116:263–269PubMedGoogle Scholar
  84. 83.
    Podack ER, Kolb WP, Esser AF, Müller-Eberhard HJ (1979) Structural similarities between C6 and C7 of human complement. J Immunol 123:1071–1077PubMedGoogle Scholar
  85. 84.
    Kolb WP, Müller-Eberhard HJ (1976) The membrane attack complex of complement: the three polypeptide chain structure of the eighth component (C8). J Exp Med 143:1131–1139Google Scholar
  86. 85.
    Steckel EW, York RG, Monahan JB, Sodetz JM (1980) The eighth component of human complement. Purification and physicochemical characterization of its unusual subunit structure. J Biol Chem 255:11997–12005PubMedGoogle Scholar
  87. 86.
    Monahan JB, Sodetz JM (1980) Binding of the eighth component of human complement of the soluble cytolytic complex is mediated by its β subunit. J Biol Chem 255:10579–10582PubMedGoogle Scholar
  88. 87.
    Monahan JB, Sodetz JM (1981) Role of the β subunit in interaction of the eighth component of human complement with the membrane-bound cytolytic complex. J Biol Chem 256:3258–3262PubMedGoogle Scholar
  89. 88.
    Zeff RA, Osika RA, Osika-Morley CJ, Faaberg KS, Lint TF (1983) Reconstitution of C8 activity in C8 deficient sera: A Ca++ requirement for C8B chain binding. Immunobiology 164:271Google Scholar
  90. 89.
    Schultz DR, Zarco RM (1970) Inhibition of the eighth component of complement (C8) by EDTA. J Immunol 104:279–288PubMedGoogle Scholar
  91. 90.
    Biesecker G, Müller-Eberhard HJ (1980) The ninth component of human complement purification and physicochemical characterization. J Immunol 124:1291–1296PubMedGoogle Scholar
  92. 91.
    Biesecker G, Gerard C, Hugh TE (1982) An amphiphilic structure of the ninth component of human complement: evidence from analysis of fragments produced by α-thrombin. J Biol Chem 257:2584–2590PubMedGoogle Scholar
  93. 92.
    Ishida B, Wisnieski BJ, Lavine CH, Esser AF (1982) Photolabelling of a hydrophobic domain of the ninth component of human complement. J Biol Chem 257:10551–10553PubMedGoogle Scholar
  94. 93.
    Kolb WP, Müller-Eberhard HJ (1974) Mode of action of human C9: Adsorption of multiple C9 molecules to cell bound C8. J Immunol 113:479–488PubMedGoogle Scholar
  95. 94.
    Ware CF, Kolb WP (1981) Assembly of the functional membrane attack complex of human complement: formation of disulfide linked C9 dimers. Proc Natl Acad Sci USA 78:6426–6430PubMedGoogle Scholar
  96. 95.
    Podack ER, Tschopp J (1982) Polymerization of the ninth component of complement: Formation of poly (C9) with a tubular ultrastructure resembling the membrane attack complex of complement. Proc Natl Acad Sci USA 79:574–578PubMedGoogle Scholar
  97. 96.
    Yamamoto KI, Migita S (1983) Mechanisms for the spontaneous formation of covalently linked polymers of the terminal membranolytic complement component (C9). J Biol Chem 258:7887–7889PubMedGoogle Scholar
  98. 97.
    Falk RJ, Dalmasso AP, Kim Y, Tsai CH, Scheinman JÏ, Gewurz H, Michael AF (1983) Neoantigen of the polymerized ninth component of complement: characterization of a monoclonal antibody and histochemical localization in renal disease. J Clin Invest 72:560–573PubMedGoogle Scholar
  99. 98.
    Douglas GN (1983) Complement system. Calbiochem-Behring, La Jolla, Ca pp 1–28Google Scholar
  100. 99.
    Nelson RA, Brebner E (1974) Isoelectric focusing of components of the complement system and certain related proteins in human serum. Immunol Commun 3:109–132PubMedGoogle Scholar
  101. 1.
    Austen KF, Becker EL, Borsos T, Lachmann PJ, Lepow IH, Mayer MM, Müller-Eberhard HJ, Nelson RA, Rapp HJ, Rosen FS, Trnka Z (1968) Nomenclature of complement. Bull WHO 39:935–938Google Scholar
  102. 1a.
    Ferrata A (1907) Die Unwirksamkeit der komplexen Hämolysine in salzfreien Lösungen und ihre Ursache. Berlin Klin Wochenschr 44:366–369Google Scholar
  103. 2.
    Nelson RA, Jensen J, Gigli I, Tamura N (1966) Methods for the separation, purification, and measurement of nine components of hemolytic complement in guinea-pig serum. Immunochemistry 3:111–135PubMedGoogle Scholar
  104. 3.
    Vroon DH, Schultz DR, Zarco RM (1970) The separation of nine components and two inactivators of components of complement in human serum. Immunochemistry 7:43–61PubMedGoogle Scholar
  105. 4.
    Sargent AU, Johnson SB, Richardson AK (1976) The isolation and functional purification of the first seven components of canine hemolytic complement. Immunochemistry 13:823–829PubMedGoogle Scholar
  106. 5.
    Barta O, Nelson RA Jr, Kuo CY (1976) Separation of six bovine complement components and one inactivator. Immunol Commun 5:75–86PubMedGoogle Scholar
  107. 6.
    Hammer CH, Wirtz GH, Renfer L, Gresham HD, Tack BF (1981) Large scale isolation of functionally active components of the human complement system. J Biol Chem 256:3995–4006PubMedGoogle Scholar
  108. 7.
    Tack BF, Janatova J, Thomas ML, Harrison RA, Hammer CH (1981) The third, fourth, and fifth components of human complement: Isolation and biochemical properties. In: Lorand L (ed) Methods in enzymology. Academic, New York, pp 64–101 (Proteolytic enzymes, part C, vol 80)Google Scholar
  109. 8.
    Tamura N, Shimada A (1971) The ninth component of guinea-pig complement. Isolation and identification as an α2-globulin. Immunology 20:415–425PubMedGoogle Scholar
  110. 9.
    Hammer CH, Nicholson A, Mayer MM (1975) On the mechanism of cytolysis by complement: evidence on insertion of C5b and Cl subunits of the C5b, 6, 7 complex into phospholipid bilayers of erythrocyte membranes. Proc Natl Acad Sci USA 72:5076–5080PubMedGoogle Scholar
  111. 10.
    Hammer CH, Abramovitz AS, Mayer MM (1976) A new activity of complement component C3: cell-bound C3b potentiates lysis of erythrocytes by C5b, 6 and terminal components. J Immunol 117:830–834PubMedGoogle Scholar
  112. 11.
    Hammer CH, Shin ML, Abramovitz AS, Mayer MM (1977) On the mechanism of cell membrane damage by complement: evidence on insertion of polypeptide chains from C8 and C9 into the lipid bilayer of erythrocytes. J Immunol 119:1–8PubMedGoogle Scholar
  113. 12.
    Borsos T, Rapp HJ (1967) Immune hemolysis: a simplified method for the preparation of EAC’4 with guinea pig or with human complement. J Immunol 99:263–268PubMedGoogle Scholar
  114. 13.
    Borsos T, Rapp HJ, Cook CT (1961) Studies on the second component of complement. III. Separation of the second component from guinea pig serum by chromatography on cellulose derivatives. J Immunol 87:330–336Google Scholar
  115. 14.
    Gaither TA, Alling DW, Frank MM (1974) A new one-step method for the functional assay of the fourth component (C4) of human and guinea pig complement. J Immunol 113:574–583PubMedGoogle Scholar
  116. 15.
    Steinbuch M, Audran R (1969) The isolation of IgG from mammalian sera with the aid of caprylic acid. Arch Biochem Biophys 134:279–284PubMedGoogle Scholar
  117. 16.
    March SC, Parikh I, Cuatrecasas P (1974) A simplified method for cyanogen bromide activation of agarose for affinity chromatography. Anal Biochem 60:149–152PubMedGoogle Scholar
  118. 17.
    Deutsch DG, Mertz ET (1970) Plasminogen purification from human plasma by affinity chromatography. Science 170:1095–1097PubMedGoogle Scholar
  119. 18.
    Tack BF, Prahl JW (1976) Third component of human complement: purification from plasma and physicochemical characterization. Biochemistry 15:4513–4521PubMedGoogle Scholar
  120. 19.
    Bolotin C, Morris S, Tack B, Prahl J (1977) Purification and structural analysis of the fourth component of human complement. Biochemistry 16:2008–2015PubMedGoogle Scholar
  121. 20.
    Nilsson UR, Tomar RH, Taylor FB Jr (1972) Additional studies on human C5: development of a modified purification method and characterization of the purified product by polyacrylamide gel electrophoresis. Immunochemistry 9:709–723PubMedGoogle Scholar
  122. 21.
    Nilsson UR, Müller-Eberhard HJ (1965) Isolation of β 1F-globulin from human serum and its characterization as the fifth component of complement. J Exp Med 122:277–298PubMedGoogle Scholar
  123. 22.
    Harrison RA, Lachmann PJ (1979) An improved purification procedure for the third component of complement and β 1H globulin from human serum. Mol Immunol 16:767–776PubMedGoogle Scholar
  124. 23.
    Shimada K, Mayumi M, Seline T, Nishioka K (1972) An improved method for separation of the fourth component of complement and C4 inactivating substance Jpn J Exp Med 42:423–434PubMedGoogle Scholar
  125. 24.
    Berger M, Gaither TA, Hammer CH, Frank MM (1981) Lack of binding of human C3, in its native state, to C3b receptors. J Immunol 127:1329–1334PubMedGoogle Scholar
  126. 25.
    Biesecker G, Müller-Eberhard HJ (1980) The ninth component of human complement: purification and physicochemical characterization. J Immunol 124:1291–1296PubMedGoogle Scholar
  127. 26.
    Reboul A, Thielens N, Villiers MB, Colomb MG (1979) Purification of human complement subcomponent C4. FEBS Lett 103:156–161PubMedGoogle Scholar
  128. 27.
    Mayer MM, Miller JA, Shin HS (1970) A specific method for purification of the second component of guinea pig complement and a chemical evaluation of the one-hit theory. J Immunol 105:327–341PubMedGoogle Scholar
  129. 28.
    Thielens NM, Villiers MB, Reboul A, Villiers CL, Colomb MG (1982) Human complement subcomponent C2: purification and proteolytic cleavage in fluid phase by #. FEBS Lett 141:19–24PubMedGoogle Scholar
  130. 29.
    Kerr MA, Porter RR (1978) The purification and properties of the second component of human complement. Biochem J 171:99–107PubMedGoogle Scholar
  131. 30.
    Whaley K, Ruddy S (1976) Modulation of the alternative complement pathway by β1H globulin. J Exp Med 144:1147–1163PubMedGoogle Scholar
  132. 31.
    Rauterberg EW, Hänsch G, Rother U (1979) Isolation of late complement components by affinity chromatography. II. Purification of the human complement component C6. Immunobiology 156:142–152PubMedGoogle Scholar
  133. 32.
    Kolb WP, Kolb LM, Savary JR (1982) Biochemical characterization of the sixth component (C6) of human complement. Biochemistry 21:294–301PubMedGoogle Scholar
  134. 33.
    Podack ER, Kolb WP, Müller-Eberhard HJ (1976) Purification of the sixth and seventh component of human complement without loss of hemolytic activity. J Immunol 116:263–269PubMedGoogle Scholar
  135. 34.
    Discipio RG, Gagnon J (1982) Characterization of human complement components C6 and C7. Mol Immunol 19:1425–1431PubMedGoogle Scholar
  136. 35.
    Tamura N, Nelson RA Jr (1968) The purification and reactivity of the first component of complement from guinea pig, human and canine sera. J Immunol 101:1333–1345PubMedGoogle Scholar
  137. 36.
    Gigli I, Porter RR, Sim RB (1976) The unactivated form of the first component of human complement, C1. Biochem J 157:541–548PubMedGoogle Scholar
  138. 37.
    Sim RB (1981) The first component of human complement-C1. Methods Enzymol 80:6–16PubMedGoogle Scholar
  139. 38.
    Medicus RG, Chapuis RM (1980) The first component of complement. I. Purification and properties of native C1. J Immunol 125:390–395PubMedGoogle Scholar
  140. 39.
    Assimeh SN, Bing DH, Painter RH (1974) A simple method for the isolation of the subcomponents of the first component of complement by affinity chromatography. J Immunol 113:225–234PubMedGoogle Scholar
  141. 40.
    Bing DH, Andrews JM, Morris KM, Cole E, Irish V (1980) Purification of subcomponents C1q, C1r, and C1s of the first component of complement from Cohn Fraction 1 by affinity chromatography. Prep Biochem 10:269–296PubMedGoogle Scholar
  142. 41.
    Arlaud GJ, Sim RB, Duplaa M, Colomb MG (1979) Differential elution of C1q, C1r, and C1s from human Cl bound to immune aggregates. Use in the rapid purification of Cl subcomponents. Mol Immunol 16:445–450PubMedGoogle Scholar
  143. 42.
    von Zeipel G, Hanson HS, von Stedingk LV (1977) Purification from euglobulin of the first component of complement and its subcomponents by heparin-Sepharose chromatography. Acta Pathol Microbiol Scand 85:123–130Google Scholar
  144. 43.
    Chapuis RM, Isliker H, Assimeh SN (1977) Studies on the precursor form of the first component of complement. I. Isolation of the proenzyme forms of C1r and C1s. Immunochemistry 14:313–317PubMedGoogle Scholar
  145. 44.
    Reid KBM (1981) Preparation of human C1q, a subcomponent of the first component of the classical pathway of complement. Methods Enzymol 80:16–25Google Scholar
  146. 45.
    Tenner AJ, Lesavre PH, Cooper NR (1981) Purification and radiolabelling of human C1q. J Immunol 127:648–653PubMedGoogle Scholar
  147. 46.
    Kolb WP, Kolb LM, Podack ER (1979) C1q: Isolation from human serum in high yield by affinity chromatography and development of a highly sensitive hemolytic assay. J Immunol 122:2103–2111PubMedGoogle Scholar
  148. 47.
    McKay EJ (1981) A simple two-step procedure for the purification of plasma C1q from different animal species. Immunol Let 3:303–308Google Scholar
  149. 48.
    Haines AL, Lepow IH (1964) Studies on human C’1-esterase. II. Function of purified C’1-esterase in the human complement system. J Immunol 92:468–478PubMedGoogle Scholar
  150. 49.
    de Bracco ME, Stroud RM (1971) C1r, subunit of the first complement component: purification, properties, and assay based on its linking role. J Clin Invest 50:838–848PubMedGoogle Scholar
  151. 50.
    Okamura K, Muramatu M, Fujii S (1973) Purification of C1s, a subunit of the first component of complement from human plasma. Biochim Biophys Acta 295:252–257PubMedGoogle Scholar
  152. 51.
    Sakai K, Stroud RM (1973) Purification, molecular properties, and activation of Cl proesterase, C1s. J Immunol 110:1010–1019Google Scholar
  153. 52.
    Valet G, Cooper NR (1974) Isolation and characterization of the proenzyme form of the C1s subunit of the first complement component. J Immunol 112:339–350PubMedGoogle Scholar
  154. 53.
    Valet G, Cooper NR (1974) Isolation and characterization of the proenzyme form of the first complement component. J Immunol 112:1667–1673PubMedGoogle Scholar
  155. 54.
    Sim RB (1981) The human complement system serine proteases C1r and C1s and their proenzymes. Methods Enzymol 80:26–42PubMedGoogle Scholar
  156. 55.
    Kerr MA, Gagnon J (1982) The purification and properties of the second component of guinea-pig complement. Biochem J 205:59–67PubMedGoogle Scholar
  157. 56.
    Polley MJ, Müller-Eberhard HJ (1968) The second component of human complement: its isolation, fragmentation by C’1 esterase, and incorporation into C’3 convertase. J Exp Med 128:533–551PubMedGoogle Scholar
  158. 57.
    Nagasawa S, Stroud RM (1977) Cleavage of C2 by C1s into the antigenically distinct fragments C2a and C2b: demonstration of binding of C2b and C4b. Proc Natl Acad Sci USA 74:2998–3001PubMedGoogle Scholar
  159. 58.
    Kerr MA (1979) Limited proteolysis of complement components C2 and factor B. Biochem J 183:615–622PubMedGoogle Scholar
  160. 59.
    Ngan B-Y, Minta JO (1981) Proteolysis of C2 and factor B: analyses of cleavage products by one- and two-dimensional peptide mapping. Mol Immunol 18:1035–1047PubMedGoogle Scholar
  161. 60.
    Pâques EP (1980) Isolation and partial characterization of the third component of the complement from porcine serum (C3) and of a crystallizable degradation product of the component of the complement system from human serum (C4). Hoppe Seylers Z Physiol Chem 361:445–456PubMedGoogle Scholar
  162. 61.
    Jacobse-Geels HEL, Daha MR, Horzinek MC (1980) Isolation and characterization of feline C3 and evidence for the immune complex pathogenesis of feline infectious peritonitis. J Immunol 125:1606–1610PubMedGoogle Scholar
  163. 62.
    Daha MR, Stuffers-Heiman M, Kijlstra A, Van Es LA (1979) Isolation and characterization of the third component of rat complement. Immunology 36:63–70PubMedGoogle Scholar
  164. 63.
    Davrinche C, Rivat C, Rivat-Peran L (1980) An improved three-step method for the purification of the third component of human complement. J Immunol Methods 35:353–359PubMedGoogle Scholar
  165. 64.
    Kunkel SL, Kreutzer DL, Goralnick S, Ward PA (1980) Purification of the third and fifth components of human complement: application of hydrophobic chromatography. J Immunol Methods 35:337–351PubMedGoogle Scholar
  166. 65.
    Thomas ML, Tack BF (1983) Identification and alignment of a thiol ester site in the third component of guinea pig complement. Biochemistry 22:942–947PubMedGoogle Scholar
  167. 66.
    Gigli I, von Zabern I, Porter RR (1977) The isolation and structure of C4, the fourth component of human complement. Biochem J 165:439–446PubMedGoogle Scholar
  168. 67.
    Lundwall A, Malmheden I, Stalenheim G, Sjoquist J (1981) Isolation of component C4 of human complement and its polypeptide chains. Eur J Biochem 117:141–146PubMedGoogle Scholar
  169. 68.
    Chan PCY, Cebra JJ (1968) Isolation and purification of the fourth component of guinea- pig complement. Immunochemistry 5:17–22PubMedGoogle Scholar
  170. 69.
    Burge J, Nicholson-Weller A, Austen KF (1981) Isolation of the fourth component of guinea pig complement and its single polypeptide chain precursor from plasma. Mol Immunol 18:47–54PubMedGoogle Scholar
  171. 70.
    Booth NA, Campbell D, Fothergill JE (1979) The purification and characterization of bovine C4, the fourth component of complement. Biochem J 177:959–965PubMedGoogle Scholar
  172. 71.
    Daha MR, van Es LA (1979) Isolation of the fourth component (C4) of rat complement. J Immunology 123:2261–2264Google Scholar
  173. 72.
    Tack BF, Morris SC, Prahl JW (1979) Fifth component of human complement: purification from plasma and polypeptide chain structure. Biochemistry 18:1490–1497PubMedGoogle Scholar
  174. 73.
    Wetsel RA, Jones MA, Kolb WP (1980) Immunoadsorbent affinity purification of the fifth component (C5) of human complement and development of a highly sensitive hemolytic assay. J Immunol Methods 35:319–335PubMedGoogle Scholar
  175. 74.
    Dessauer A, Rother U (1983) The fifth component of complement (C5): purification without activation. Immunobiology 164:370–379PubMedGoogle Scholar
  176. 75.
    Linscott WD, Nishioka K (1963) Components of guinea pig complement. II. Separation of serum fractions essential for immune hemolysis. J Exp Med 118:795–815PubMedGoogle Scholar
  177. 76.
    Cook CC, Shin HS, Mayer MM, Laudenslayer KA (1971) The fifth component of the guinea pig complement system. J Immunol 106:467–472PubMedGoogle Scholar
  178. 77.
    Kinoshita T, Hong K, Kondo K, Inoue K (1981) Fifth component of guinea pig complement: purification and characterization. J Immunol 126:2414–2418PubMedGoogle Scholar
  179. 78.
    Nilsson U (1967) Separation and partial purification of the sixth, seventh and eighth components of human haemolytic complement. Acta Pathol Microbiol Immunol Scand 70:469–480Google Scholar
  180. 79.
    Arroyave CM, Müller-Eberhard HJ (1971) Isolation of the sixth component of complement from human serum. Immunochemistry 8:995–1006PubMedGoogle Scholar
  181. 80.
    Podack ER, Kolb WP, Esser AF, Müller-Eberhard HJ (1979) Structural similarities between C6 and C7 of human complement. J Immunol 123:1071–1077PubMedGoogle Scholar
  182. 81.
    Arroyave CM, Müller-Eberhardt HJ (1973) Isolation of the C system from human serum. J Immunol (Abstract) 111:302–303Google Scholar
  183. 82.
    Manni JA, Müller-Eberhard HJ (1969) The eighth component of human complement (C8): isolation, characterization, and hemolytic efficiency. J Exp Med 130:1145–1160PubMedGoogle Scholar
  184. 83.
    Kolb WP, Müller-Eberhard HJ (1976) The membrane attack mechanism of complement: the three polypeptide chain structure of the eighth component (C8). J Exp Med 143:1131–1139Google Scholar
  185. 84.
    Steckel EW, York RG, Monahan JB, Sodetz JM (1980) The eighth component of human complement. Purification and physicochemical characterization of its unusual subunit structure. J Biol Chem 255:11997–12005PubMedGoogle Scholar
  186. 85.
    Kinoshita T, Hong K, Kondo K, Inoue K (1982) Eighth component of guinea pig complement: purification and characterization. Mol Immunol 19:589–597PubMedGoogle Scholar
  187. 86.
    Hadding U, Müller-Eberhard HJ (1969) The ninth component of human complement: Isolation, description and mode of action. Immunology 16:719–735PubMedGoogle Scholar
  188. 87.
    Rauterberg EW, Schieck C, Hänsch G (1979) Isolation of late complement components by affinity chromatography: I. Purification of the human complement component C9 and production of a C9-defective human serum. Z Immun Forsch 155:365–377Google Scholar
  189. 1.
    Ackerman SK, Friend PS, Hoidal JR, Douglas SD (1978) Production of C2 by human alveolar macrophages. Immunology 35:369–372PubMedGoogle Scholar
  190. 2.
    Al-Adnani MS, McGee JOD (1976) C1q production and secretion by fibroblasts. Nature 263:145–146PubMedGoogle Scholar
  191. 3.
    Almeda S, Rosenberg RD, Bing DH (1983) The binding properties of human complement component C1q. J Biol Chem 258:784–791Google Scholar
  192. 4.
    Alper CA (1981) Complement and the MHC. In: Dorf ME (ed) The role of the major histocompatibility complex in immunobiology. Garland, New York, pp 173–220Google Scholar
  193. 5.
    Alper CA, Johnson AM, Birtch AG, Moore RD (1969) Human C’3: evidence for the liver as the primary site of synthesis. Science 163:286–288PubMedGoogle Scholar
  194. 6.
    Alper CA, Marcus D, Raum D, Petersen BH, Spira TJ (1983) Genetic polymorphism in C8 beta-chains. J Clin Invest 72:1526–1531PubMedGoogle Scholar
  195. 7.
    Alper CA, Raum D, Awdeh Z, Petersen BH, Taylor PD, Starzl TE (1980) Studies of hepatic synthesis in vivo of plasma proteins including orosomucoid, transferrin, alpa-1-antitrypsin, C8 and factor B. Clin Immunol Immunopathol 16:84–89PubMedGoogle Scholar
  196. 8.
    Alpert SE, Auerbach HS, Cole FS, Colten HR (1983) Macrophage maturation: differences in complement secretion by marrow, monocyte, and tissue macrophages detected with an improved hemolytic plaque assay. J Immunol 130:102–107PubMedGoogle Scholar
  197. 9.
    Atkinson JP, McGinnis K, Brown L, Peterein J, Shreffler D (1980) A murine C4 molecule with reduced hemolytic efficiency. J Exp Med 151:492–497PubMedGoogle Scholar
  198. 10.
    Atkinson JP, Shin H, Frank MM (1974) Metabolic behavior of C3 in normal, C4-deficient (C4D) and cortisone treated guinea pigs. J Immunol 113:1085–1090PubMedGoogle Scholar
  199. 11.
    Auerbach HS, Baker RD, Matthews WJ, Colten HR (1984) Molecular mechanism for feedback regulation of C4 biosynthesis in guinea pig peritoneal macrophages. J Exp Med 159:1750–1761PubMedGoogle Scholar
  200. 12.
    Auerbach HS, Lalande ME, Latt S, Colten HR (1983) Isolation of guinea pig macrophages bearing surface C4 by fluorescence activated cell sorting: correlation between surface C4 antigen and C4 protein secretion. J Immunol 131:2420–2426PubMedGoogle Scholar
  201. 13.
    Barber TA, Burkholder PM (1978) Enumeration and ultrastructure of C4 producing free alveolar cells from guinea pig lung. J Immunol 120:716–725PubMedGoogle Scholar
  202. 14.
    Baltz ML, Gomer K, Davies AJS, Evans DJ, Klaus GGB, Pepys MB (1980) Differences in the acute phase responses of serum amyloid P-complement (SAP) and C3 to injections of casein or bovine serum albumin in amyloid-susceptible and -resistant mouse strains. Clin Exp Immunol 39:355–360PubMedGoogle Scholar
  203. 15.
    Bates CJ, Levene CI, Oldroyd RG, Lachmann PJ (1978) Complement component C1q is insensitive to acute vitamin C deficiency in guinea pigs. Biochemin Biophys Acta 540:423–430Google Scholar
  204. 16.
    Beatty DW, Davis AE, Cole FS, Einstein LP, Colten HR (1981) Biosynthesis of complement by human monocytes. Clin Immunol Immunopathol 18:334–343PubMedGoogle Scholar
  205. 17.
    Bentley CD, Bitter-Suermann D, Hadding U, Brade V (1976) In vitro synthesis of factor B of the alternative pathway of complement by mouse peritoneal macrophages. Eur J Immunol 6:393–398PubMedGoogle Scholar
  206. 18.
    Bentley C, Fries W, Brade V (1978) Synthesis of factors D, B and P of the alternative pathway of complement activation, as well as of C3, by guinea pig peritoneal macrophages in vitro. Immunology 35:971–980PubMedGoogle Scholar
  207. 19.
    Bing DH, Spurlock SE, Bern MM (1975) Synthesis of the first component of complement by primary cultures of human tumors of the colon and urogenital tract and comparable normal tissue. Clin Immunol Immunopathol 4:341–351PubMedGoogle Scholar
  208. 20.
    Bitter-Suermann D, Hoffman T, Burger R, Hadding U (1981) Linkage of total deficiency of the second component (C2) of the complement system and of genetic polymorphism to the major histocompatibility complex of the guinea pig. J Immunol 127:608–612PubMedGoogle Scholar
  209. 21.
    Bitter-Suermann D, Kronke M, Brade V, Hadding U (1977) Inherited polymorphism of guinea pig factor B and C4: evidence for genetic linkage between the C4 and Bf loci. J Immunol 118:1822–1826PubMedGoogle Scholar
  210. 22.
    Bordin S, Kolb WP, Page RC (1983) C1q receptors on cultured human gingival fibroblasts: analysis of binding properties. J Immunol 130:1871–1875PubMedGoogle Scholar
  211. 23.
    Borsos T, Loos M, Chapuis RM, Medicus R, Isliker H (1980) A novel way of relating the structure of C1q to the hemolytic activity of the first component of complement. Mol Immunol 17:1415–1421PubMedGoogle Scholar
  212. 24.
    Brade V, Damerau B, Kreuzpaintner G (1981) Studies on the influence of C3 fragments on the secretory function and viability of guinea pig peritoneal macrophages. In: Forster O, Landy M (eds), Heterogeneity of mononuclear phagocytes. Academic, New York, pp 270–277Google Scholar
  213. 25.
    Brade V, Hall RE, Colten HR (1977) Biosynthesis of pro-C3, a precursor of the third component of complement. J Exp Med 146:759–765PubMedGoogle Scholar
  214. 26.
    Brade V, Kreuzpaintner G (1982) Functional C4 active complement components secreted by guinea pig peritoneal macrophages. Immunobiology 161:315–321PubMedGoogle Scholar
  215. 27.
    Burton DR, Boyd J, Brampton AD, Easterbrook-Smith SB, Emanuel EJ, Novotny J, Rademacher TW, van Schravendijk MR, Sternberg MJE, Dwek RA (1980) The C1q receptor site on immunoglobulin G. Nature 288:338–344PubMedGoogle Scholar
  216. 28.
    Carroll MC, Campbell DR, Bentley DR, Porter RR (1984) A molecular map of the human major histocompatibility complex class III region linking complement genes C4, C2 and factor B. Nature 307:237–241PubMedGoogle Scholar
  217. 29.
    Carroll MC, Capra JD (1979) Studies on murine Ss-protein: demonstration that S locus encodes the structural gene for fourth component of complement. Proc Natl Acad Sci USA 76:4641–4645PubMedGoogle Scholar
  218. 30.
    Carroll MC, Porter RR (1983) Cloning of a human complement component C4 gene. Proc Natl Acad Sci USA 80:264–267PubMedGoogle Scholar
  219. 31.
    Chan AC, Mitchell KR, Munns TW, Karp DR, Atkinson JP (1983) Identification and partial characterization of the secreted form of the fourth component of human complement: evidence that it is different from the major plasma form. Proc Natl Acad Sci USA 80:268–272PubMedGoogle Scholar
  220. 32.
    Chaplin DD, Woods DW, Whitehead AS, Goldberger G, Colten HR, Seidman JG (1983) Molecular map of the murine S region. Proc Natl Acad Sci USA 80:6947–6951PubMedGoogle Scholar
  221. 33.
    Chapuis RM, Hauptmann G, Grosshans E, Isliker H (1982) Structural and functional studies in C1q deficiency. J Immunol 129:1509–1512PubMedGoogle Scholar
  222. 34.
    Christie DL, Gagnon J (1983) Amino acid sequence of the Bb fragment from complement factor B. Biochem J 209:61–70PubMedGoogle Scholar
  223. 35.
    Cole FS, Auerbach HS, Goldberger G, Colten HR (1983) Mechanisms regulating complement production during monocyte-macrophage maturation. Immunobiol 164:224Google Scholar
  224. 36.
    Cole FS, Matthews WJ, Marino JT, Gash DJ, Colten HR (1980) Control of complement synthesis and secretion in bronchoalveolar and peritoneal macrophages. J Immunol 125:1120–1124PubMedGoogle Scholar
  225. 37.
    Cole FS, Matthews WJ, Rossing TH, Gash DJ, Lichtenberg NA, Pennington JE (1983) Complement biosynthesis by human bronchoalveolar macrophages. Clin Immunol Immunopathol 27:153–159PubMedGoogle Scholar
  226. 38.
    Cole FS, Schneeberger EE, Lichtenberg NA, Colten HR (1982) Complement biosynthesis in human breast milk macrophages and blood monocytes. Immunol 46:429–441Google Scholar
  227. 39.
    Colten HR (1972) In Vitro synthesis of a regulator of mammalian gene expression. Proc Natl Acad Sci USA 69:2233–2236PubMedGoogle Scholar
  228. 40.
    Colten HR (1972) Ontogeny of the human complement system: in vitro biosynthesis of individual complement components by fetal tissues. J Clin Invest 51:725–730PubMedGoogle Scholar
  229. 41.
    Colten HR (1976) Biosynthesis of complement. Adv Immunol 22:67–118PubMedGoogle Scholar
  230. 42.
    Colten HR (1982) Biosynthesis of the MHC-linked complement proteins (C2, C4, and factor B) by mononuclear phagocytes. Mol Immunol 19:1279–1285PubMedGoogle Scholar
  231. 43.
    Colten HR (1983) Molecular genetics of the major histocompatibility linked complement genes. Springer Semin Immunopathol 6:149–158PubMedGoogle Scholar
  232. 44.
    Colten HR, Alper CA, Rosen FS (1981) Genetics and biosynthesis of complement proteins. N Engl J Med 304:653–656PubMedGoogle Scholar
  233. 45.
    Colten HR, Borsos T, Rapp HJ (1966) In vitro synthesis of the first component of complement by guinea pig small intestine. Proc Natl Acad Sci USA 56:1158–1163PubMedGoogle Scholar
  234. 46.
    Colten HR, Einstein LP (1976) Complement metabolism: cellular and humoral regulation. Trans Rev 32:3–11Google Scholar
  235. 47.
    Colten HR, Frank MM (1972) Biosynthesis of the second and fourth components of complement in vitro by tissues isolated from guinea pigs with genetically determined C4 deficiency. Immunol 22:991–999Google Scholar
  236. 48.
    Colten HR, Goldberger G, Kay RM, Woods DE (1983) Biosynthesis, processing and secretion of biologically active complement proteins by Xenopus oocytes injected with human mRNA. Immunobiology 164:225–226Google Scholar
  237. 49.
    Colten HR, Gordon JM, Borsos T, Rapp HJ (1968) Synthesis of the first component of human complement in vitro. J Exp Med 128:595–604PubMedGoogle Scholar
  238. 50.
    Colten HR, Gordon JM, Rapp HJ, Borsos T (1968) Synthesis of the first component of guinea pig complement by columnar epithelial cells of the small intestine. J Immunol 100:788–792PubMedGoogle Scholar
  239. 51.
    Colten HR, Ooi YM, Edelson PJ (1979) Synthesis and secretion of complement proteins by macrophages. Ann NY Acad Sci 332:482–490PubMedGoogle Scholar
  240. 52.
    Colten HR, Parkman R (1972) Biosynthesis of C4 (fourth component of complement) by hybrids of C4-deficient guinea pig cells and HeLa cells. Science 176:1029–1031PubMedGoogle Scholar
  241. 53.
    Cooper NR (1983) Activation and regulation of the first complement component. Fed Proc 42:134–137PubMedGoogle Scholar
  242. 54.
    DaSilva FP, Hoecker GE, Day NK, Vienne D, Rubinstein P (1978) Murine complement component 3: genetic variation and linkage to H-2. Proc Natl Acad Sci USA 75:963–965Google Scholar
  243. 55.
    Day NK, Gewurz H, Pickering RJ, Good RA (1970) Ontogenetic development of C1q synthesis in the piglet. J Immunol 104:1316–1319PubMedGoogle Scholar
  244. 56.
    DeCeulaer C, Papazoglou S, Whaley K (1980) Increased biosynthesis of complement components by cultured monocytes, synovial fluid macrophages and synovial membrane cells from patients with rheumatoid arthritis. Immunology 41:37–43Google Scholar
  245. 57.
    Domdey H, Wiebauer K, Kazmaier M, Muller V, Odink K, Fey G (1982) Characterization of the mRNA and cloned cDNA specifying the third component of mouse complement. Proc Natl Acad Sci USA 79:7619–7623PubMedGoogle Scholar
  246. 58.
    Einstein LP, Alper CA, Bloch KJ, Herrin JT, Rosen FS, David JR, Colten HR (1975) Biosynthetic defect in monocytes from human beings with genetic deficiency of the second component of complement. N Engl J Med 292:1169–1171PubMedGoogle Scholar
  247. 59.
    Einstein LP, Hansen PJ, Ballow M, Davis AE, Davis JS, Alper CA, Rosen FS, Colten HR (1977) Biosynthesis of the third component of complement (C3) in vitro by monocytes from both normal and homozygous C3-deficient humans. J Clin Invest 60:963–969PubMedGoogle Scholar
  248. 60.
    Einstein LP, Schneeberger EE, Colten HR (1976) Synthesis of the second component of complement by long-term primary cultures of human monocytes. J Exp Med 143:114–126PubMedGoogle Scholar
  249. 61.
    Ellman L, Green I, Frank MM (1970) Genetically controlled total deficiency of the fourth component of complement in the guinea pig. Science 170:74–75PubMedGoogle Scholar
  250. 62.
    Ezekowitz RAB, Sim RB, Hill M, Gordon S (1984) Local opsonisation by secreted macrophage complement components. Role of receptors for complement in uptake of zymosan. J Exp Med 159:244–260PubMedGoogle Scholar
  251. 63.
    Ferreira A, Michaelson J, Nussenzweig V (1980) A polymorphism of the gamma-chain of mouse C4 controlled by the S region of the major histocompatibility complex. J Immunol 125:1178–1182PubMedGoogle Scholar
  252. 64.
    Fey G, Colten HR (1981) Biosynthesis of complement components. Fed Proc 40:2099–2104PubMedGoogle Scholar
  253. 65.
    Fey G, Domdey H, Wiebauer K, Whitehead AS, Odink K (1983) Structure and expression of the C3 gene. Springer Semin Immunopathol 6:119–147PubMedGoogle Scholar
  254. 66.
    Fey G, Odink K, Chapuis RM (1980) Synthesis of the mouse complement component C4 (Ss-protein) by peritoneal macrophages: kinetics of secretion and glycosylation of the subunits. Eur J Immunol 10:75–82PubMedGoogle Scholar
  255. 67.
    Ferreira A, Nussenzweig V, Gigli I (1978) Structural and functional differences between the H-2 controlled Ss and Slp proteins. J Exp Med 148:1186–1197PubMedGoogle Scholar
  256. 68.
    Fleming KA, McGee JOD (1982) The relationship between collagen and C1q biosynthesis in cultured human fibroblasts. Biochem J 206:287–293PubMedGoogle Scholar
  257. 69.
    Fleming KA, Reid KBM, McGee JOD (1983) An assay for C1q biosynthesis in cultured human fibroblasts. J Immunol Methods 56:43–54PubMedGoogle Scholar
  258. 70.
    Fu SM, Kunkel HG, Brusman HP, Allen FH, Fotino M (1974) Evidence for linkage between HL-A histocompatibility genes and those involved in the synthesis of the second component of complement. J Exp Med 140:1108–1111PubMedGoogle Scholar
  259. 71.
    Gagnon J, Christie DL (1983) Amino acid sequence of the Bb fragment from human complement factor B. Biochem J 209:51–60PubMedGoogle Scholar
  260. 72.
    Geiger H, Day NKB, Good RA (1972) Ontogenetic development and synthesis of hemolytic C8 by piglet tissues. J Immunol 108:1092–1097PubMedGoogle Scholar
  261. 73.
    Germain RN, Norcross MA (1983) Functional expression of a transfected murine class II MHC gene. Nature 306:190–194PubMedGoogle Scholar
  262. 74.
    Gigli I (1978) A single chain precursor of human C4 in serum. Nature 272:836–837PubMedGoogle Scholar
  263. 75.
    Gitlin D, Biasucci A (1969) Development of γG, γA, γM, β1C/β1A, C1 esterase inhibitor, ceruloplasmin, transferrin, hemopexin, haptoglobin, fibrinogen, plasminogen, alpha-1-antitrypsin, orosomucoid, beta-lipoprotein, alpha-2macroglobulin, and prealbumin in the human conceptus. J Clin Invest 48:1433–1442PubMedGoogle Scholar
  264. 76.
    Goldberger G, Abraham GN, Williams J, Colten HR (1980) Primary structure of pro-C4, the precursor of the fourth component of guinea pig complement. J Biol Chem 255:7071–7074PubMedGoogle Scholar
  265. 77.
    Goldberger G, Arnaout MA, Aden D, Kay R, Rits M; Colten HR (1984) Biosynthesis and postsynthetic processing of human C3b/C4b inactivator (factor I) in three hepatoma cell lines. J Biol Chem 259:6492–6497PubMedGoogle Scholar
  266. 78.
    Goldberger G, Brenner MJ, Colten HR (1979) Kinetics of synthesis and secretion of a precursor of the fourth component of complement. Fed Proc 38:1011Google Scholar
  267. 79.
    Goldberger G, Cole FS, Einstein LP, Auerbach HS, Bitter-Suermann D, Colten HR (1982) Biosynthesis of a structurally abnormal C2 complement protein by macrophages from C2 deficient guinea pigs. J Immunol 129:2061–2065PubMedGoogle Scholar
  268. 80.
    Goldberger G, Colten HR (1980) Precursor complement protein (pro-C4) is converted in vitro to native C4 by plasmin. Nature 286:514–516PubMedGoogle Scholar
  269. 81.
    Goldberger G, Thomas ML, Tack BF, Williams J, Colten HR, Abraham GN (1981) NH2-terminal structure and cleavage of guinea pig pro-C3, the precursor of the third complement component. J Biol Chem 256:12617–12619PubMedGoogle Scholar
  270. 82.
    Goldman MB, Cohen L, O’Rourke KS, Goldman JN (1982) Functional and developmental differences between genetically controlled phenotypes of the sixth component of complement in rabbits. J Immunol 129:180–183PubMedGoogle Scholar
  271. 83.
    Goldman MB, Cohen L, Stronski K, Bangalone S, Goldman JN (1982) Genetic control of C6 polymorphism and C6 deficiency in rabbits. J Immunol 128:43–48PubMedGoogle Scholar
  272. 84.
    Goldman MB, O’Rourke KS, Goldman JN (1982) Antibody-induced suppression and post-supression stimulation of complement in vitro. I. Effects of anti-C4 on cultured guinea pigs peritoneal cells. Cell Immunol 70:118–131PubMedGoogle Scholar
  273. 85.
    Gorski JP, Hugli TE, Muller-Eberhard HJ (1979) C4a: the third anaphylatoxin of the human complement system. Proc Natl Acad Sci USA 76:5299–5302PubMedGoogle Scholar
  274. 86.
    Hadding U, Bentley C, Bitter-Suermann D, Brade V (1976) Factor B of the properdin system is synthesized by mouse peritoneal macrophages in vitro. Fed Proc 35:654Google Scholar
  275. 87.
    Halbluachs L, Lachmann PJ (1976) Factor B of the alternative complement pathway on human lymphocytes. Scand J Immunol 5:697–702Google Scholar
  276. 88.
    Hall RE, Colten HR (1977) Cell-free synthesis of the fourth component of guinea pig complement (C4): identification of a precursor of serum C4 (pro-C4). Proc Natl Acad Sci USA 74:1707–1710PubMedGoogle Scholar
  277. 89.
    Hall RE, Colten HR (1977) Molecular size and subunit structure of the fourth component of guinea pig complement. J Immunol 118:1903–1905PubMedGoogle Scholar
  278. 90.
    Hall RE, Colten HR (1978) Genetic defect in biosynthesis of the precursor form of the fourth component of complement. Science 199:69–70PubMedGoogle Scholar
  279. 91.
    Hannema AJ, Kluin-Nelemans HC, Hack CE, Mallee C, van Helden HPT, Eerenber-Belmer AJM (1982) A functional deficiency of C1q in 3 family members. Mol Immunol 19:1373Google Scholar
  280. 92.
    Hartveit F, Borve W, Thunold S (1973) Serum complement levels and response to turpentine inflammation in mice. Acta Pathol Microbiol Immunol Scand [A] [Suppl] 236:54–59Google Scholar
  281. 93.
    Hugli TE (1981) The structural basis for anaphylatoxin and chemotactic functions of C3a, C4a and C5a. CRC Crit Rev Immunol 1:321–366Google Scholar
  282. 94.
    Johnson AM, Alper CA, Rosen FS, Craig JM (1971) Immunofluorescent hepatic localization of complement proteins: evidence for a biosynthetic defect in hereditary angioneurotic edema (HANE). J Clin Invest 50:50aGoogle Scholar
  283. 95.
    Karp DR (1983) Post-translational modification of the fourth component of complement. Effect of tunicamycin and amino acid analogs on the formation of the internal thiol ester and disulfide bonds. J Biol Chem 258:14490–14495PubMedGoogle Scholar
  284. 96.
    Karp DR (1983) Post-translational modification of the fourth component of complement. Sulfation of the alpha chain. J Biol Chem 258:12745–12748PubMedGoogle Scholar
  285. 97.
    Karp DR, Capra JD, Atkinson JP, Shreffler DC (1982) Structural and functional characterization of an incompletely processed form of murine C4 and Slp. Immunol 128:2336–2341Google Scholar
  286. 98.
    Karp DR, Parker KL, Shreffler DC, Slaughter C, Capra JD (1982) Amino acid sequence homologies and glycosylation differences between the fourth component of murine complement and sex-limited protein. Proc Natl Acad Sci USA 79:6347–6349PubMedGoogle Scholar
  287. 99.
    Karp DR, Shreffler DC, Atkinson JP (1982) Characterization of the Mr difference between secreted murine fourth component of complement and the major plasma form: evidence for carboxyl-terminal cleavage of the alpha chain. Proc Natl Acad Sci USA 79:6666–6670PubMedGoogle Scholar
  288. 100.
    Kerr MA, Gagnon J (1982) The purification and properties of the second component of guinea pig complement. Biochem J 205:59–67PubMedGoogle Scholar
  289. 101.
    Knobel JR, Villiger W, Isliker H (1972) Chemical analysis and electron microscopy studies of human C1q prepared by different methods. Eur J Immunol 5:78–81Google Scholar
  290. 102.
    Knowles BB, Howe CC, Aden DP (1980) Human hepatocellular carcinoma cell lines secrete the major plasma proteins and hepatitis B surface antigen. Science 209:497–499PubMedGoogle Scholar
  291. 103.
    Kohler PF (1973) Maturation of the human complement system. I.Onset time and sites of fetal C1q, C4, C3, and C5 synthesis. J Clin Invest 52:671–677PubMedGoogle Scholar
  292. 104.
    Kolski GB, Strunk RC (1981) Soybean oil emulsion induces a selective and reversible inhibition of C2 production by human mononuclear phagocytes. J Immunol 126:2267–2271PubMedGoogle Scholar
  293. 105.
    Kreuzpaintner G, Brade V (1983) Effect of cycloheximide and of anti-C3 Fab’ on the intrinsic synthesis and secretion of lysosomal enzyme and of complement components by guinea pig peritoneal macrophages. Immunology 49:337–342PubMedGoogle Scholar
  294. 106.
    Lai AF, van Furth R (1975) In vitro synthesis of some complement components (C1q, C3, and C4) by lymphoid tissues and circulating leukocytes in man. Immunology 28:359–368Google Scholar
  295. 107.
    Lally ET, McArthur WP, Baehni PC (1982) Biosynthesis of complement components in chronically inflamed gingiva. J Periodontol Res 17:257–262Google Scholar
  296. 108.
    Lappin D, Whaley K (1980) Effect of histamine on monocyte complement production. I. Inhibition of C2 production mediated by its action on H2 receptors. Clin Exp Immunol 41:497–504PubMedGoogle Scholar
  297. 109.
    Leddy JP, Frank MM, Gaither T, Baum J, Klemperer MR (1974) Hereditary deficiency of the sixth component of complement in man. I. Immunochemical, biologic and family studies. J Clin Invest 53:544–553PubMedGoogle Scholar
  298. 110.
    Lepow IH, Naff GB, Todd EW, Pensky J, Hinz CH (1963) Chromatographic resolution of the first component of human complement into three activities. J Exp Med 117:983–1008PubMedGoogle Scholar
  299. 111.
    Levy NL, Snyderman R, Ladda RL, Lieberman R (1973) Cytogenetic engineering in vivo: restoration of biologic complement activity to C5-deficient mice by intravenous inoculation of hybrid cells. Proc Natl Acad Sci USA 70:3125–3129PubMedGoogle Scholar
  300. 112.
    Littleton CD, Kessler D, Burkjolder PM (1970) Cellular basis for synthesis of the fourth component of guinea pig complement as determined by a hemolytic plaque assay. Immunology 18:691–702Google Scholar
  301. 113.
    Littman BH, Ruddy S (1977) Production of the second component of complement by human monocytes: stimulation by antigen activated lymphocytes or lymphokines. J Exp Med 145:1344–1352PubMedGoogle Scholar
  302. 114.
    Littman BH, Hall RE, Muchmore AV (1983) Lymphokine and phorbol (PMA) regulation of complement (C2) synthesis using U937. Cell Immunol 76:189–195PubMedGoogle Scholar
  303. 115.
    Loos M (1983) Biosynthesis of the collagen-like C1q molecule and its receptor functions for Fc and polyanionic molecules on macrophages. Curr Top Microbiol Immunol 102:2–56Google Scholar
  304. 116.
    Lundwall AB, Wetsel RA, Kristensen T, Whitehead AS, Woods D, Ogden RL, Colten HR, Tack BF (1985) Isolation and sequence analysis of a cDNA clone encoding the fifth component of human complement. J Biol Chem 260:2108–2112PubMedGoogle Scholar
  305. 117.
    McClelland DBL, van Furth R (1976) In vitro synthesis of beta-1-C/beta-1-A globulin (the C3 component of complement) by tissues and leucocytes of mice. Immunology 31:855–861PubMedGoogle Scholar
  306. 118.
    McPhaden AR, Lappin D, Whaley K (1982) Biosynthesis of complement components. J Clin Lab Immunol 8:1–8PubMedGoogle Scholar
  307. 119.
    McPhaden AR, Whaley K (1982) Modulation of C2 biosynthesis by antigen-antibody complexes. J Clin Lab Immunol 7:15–19PubMedGoogle Scholar
  308. 120.
    Matthews WJ, Goldberger F, Marino JT, Einstein LP, Gash DJ, Colten HR (1982) Complement proteins C2, C4, and factor B: effect of glycosylation on their secretion and catabolism. Biochem J 204:839–846PubMedGoogle Scholar
  309. 121.
    Matthews WJ, Marino JT, Goldberger G, Gash DJ, Colten HR (1979) Feedback inhibition of the biosynthesis of the fourth component of complement (C4). Fed Proc 38:1011Google Scholar
  310. 122.
    Minta JO, Ngan BY, Pang ASD (1979) Purification and characterization of a single chain precursor C3-protein (pro-C3) from normal human plasma. J Immunol 123:2415–2420PubMedGoogle Scholar
  311. 123.
    Mondino BJ, Sundar-Raj CV, Brady KJ (1982) Production of first component of complement by corneal fibroblasts in tissue culture. Arch Ophthalmol 100:478–480PubMedGoogle Scholar
  312. 124.
    Morris KM, Aden DP, Knowles BB, Colten HR (1982) Complement biosynthesis by the human hepatoma-derived cell line, Hep G2. J Clin Invest 70:906–913PubMedGoogle Scholar
  313. 125.
    Morris KM, Colten HR, Bing DH (1978) The first component complement. A quantitative comparison of its biosynthesis in culture by human epithelial and mesenchymal cells. J Exp Med 148:1007–1019PubMedGoogle Scholar
  314. 126.
    Morris KM, Goldberger G, Colten HR, Aden DP, Knowles BB (1982) Biosynthesis and processing of a human precursor complement protein, pro-C3, in a hepatoma-derived cell line. Science 215:399–400PubMedGoogle Scholar
  315. 127.
    Müller W, Hanauske-Abel H, Loos M (1978) Synthesis of the first component of complement by human and guinea pig peritoneal macrophages: evidence for an independent production of the C1 subunits. J Immunol 121:1578–1584PubMedGoogle Scholar
  316. 128.
    Müller W, Hanauske-Abel H, Loos M (1978) Reversible inhibition of C1q release from guinea pig macrophages by 2,2′-dipyridyl. FEBS Lett 90:218–222PubMedGoogle Scholar
  317. 129.
    Nagasawa S, Stroud RM (1977) Cleavage of C2 by C1s into the antigenically distinct fragments C2a and C2b: demonstration of binding of C2b to C4b, Proc Natl Acad Sci USA 74:2998–3001PubMedGoogle Scholar
  318. 130.
    Newell SL, Shreffler DC, Atkinson JP (1982) Biosynthesis of C4 by mouse peritoneal macrophages. I. Characterization of an in vitro culture system and comparison of C4 synthesis of “low” vs “high” C4 strains. J Immunol 129:653–659PubMedGoogle Scholar
  319. 131.
    Newell SL, Atkinson JP (1983) Biosynthesis of C4 by mouse peritoneal macrophages. II. Comparison of C4 synthesis by resident and elicited cell populations. J Immunol 130:834–838PubMedGoogle Scholar
  320. 132.
    Nilsson UR, Mandle RJ, McConnell-Mapes JA (1975) Human C3 and C5: subunit structure and modification by trypsin and C42-C423. J Immunol 114:815–822PubMedGoogle Scholar
  321. 133.
    Ogata RT, Shreffler DC, Sepich DS, Lilly SP (1983) cDNA clone spanning the alpha- gamma subunit junction in the precursor of the murine fourth complement component (C4). Proc Natl Acad Sci USA 80:5061–5065PubMedGoogle Scholar
  322. 134.
    Ooi YM (1982) Histamine suppressed in vitro synthesis of precursor (pro-C5) of the fifth complement component (C5) by mouse peritoneal macrophages. J Immunol 129:200–205PubMedGoogle Scholar
  323. 135.
    Ooi YM, Colten HR (1979) Biosynthesis and post-synthetic modification of a precursor (pro-C5) of the fifth component of mouse complement (C5). J Immunol 123:2494–2498PubMedGoogle Scholar
  324. 136.
    Ooi YM, Colten HR (1979) Genetic defect in secretion of complement C5 in mice. Nature 282:207–208PubMedGoogle Scholar
  325. 137.
    Ooi YM, Harris DE, Edelson PJ, Colten HR (1980) Post-translational control of complement (C5) production by resident and stimulated mouse macrophages. J Immunol 124:2077–2081PubMedGoogle Scholar
  326. 138.
    Ooi YM, Ooi BS (1982) Biosynthesis of membrane factor B by mouse peritoneal macrophages. Nature 298:389–391PubMedGoogle Scholar
  327. 139.
    Pangburn MK (1983) Activation of complement via the alternative pathway. Fed Proc 42:139–143PubMedGoogle Scholar
  328. 140.
    Parker KL, Capra JD, Shreffler DC (1980) Partial amino acid sequences of the murine fourth component of complement (C4): demonstration of homology with human C4 and identification of amino terminal subunit in pro-C4. Proc Natl Acad Sci USA 77:4275–4278PubMedGoogle Scholar
  329. 141.
    Parker KL, Carroll MC, Shreffler DC, Capra JD (1981) Identification of H-2-controlled structural variants of the murine Slp protein and demonstration of cis-regulation of its expression. J Immunol 126:995–997PubMedGoogle Scholar
  330. 142.
    Parker KL, Roos MH, Shreffler DC (1979) Structural characterization of the murine fourth component of complement and sex-limited proteins and their precursors: evidence for two loci in the S-region of the H-2 complex. Proc Natl Acad Sci USA 76:5853–5857PubMedGoogle Scholar
  331. 143.
    Passmore HC, Shreffler DC (1970) A sex-linked serum protein variant in the mouse: inheritance and association with the H-2 region. Biochem Genet 4:351–365PubMedGoogle Scholar
  332. 144.
    Patel F, Minta JO (1979) Biosynthesis of a single chain pro-C5 by normal mouse liver mRNA: analysis of the molecular basis of C5 deficiency in AKR/J mice. J Immunol 123:2408–2414PubMedGoogle Scholar
  333. 145.
    Pennington JE, Matthews WJ, Marino JT, Colten HR (1979) Cyclophosphamide and cortisone acetate inhibit complement biosynthesis by guinea pig bronchoalveolar macrophages. J Immunol 123:1318–1321PubMedGoogle Scholar
  334. 146.
    Perlmutter DH, Cole FS, Goldberger G, Colten HR (1984) Distinct primary translation products from human liver mRNA give rise to secreted and cell-associated forms of complement protein C2. J Biol Chem 259:10380–10385PubMedGoogle Scholar
  335. 147.
    Phillips MR, Rother UA, Rother KO, Thorbecke GJ (1969) Studies on the serum proteins of chimeras. III. Detection of donor type C5 in allogenic and congenic post-irradiation chimeras. Immunology 17:315–321PubMedGoogle Scholar
  336. 148.
    Porter RR, Reid KBM (1978) The biochemistry of complement. Nature 275:699–704PubMedGoogle Scholar
  337. 149.
    Porter RR, Reid KBM (1979) Activation of the complement system by antibody-antigen complexes: The classical pathway. Adv Protein Chem 33:1–71PubMedGoogle Scholar
  338. 150.
    Quastei M, Harrison R, Cicardi M, Alper CA, Rosen FS (1983) Behavior in vivo of normal and dysfunctional C1 inhibitor in normal subjects and patients with hereditary angioneurotic edema. J Clin Invest 71:1041–1046Google Scholar
  339. 151.
    Raum D, Donaldson VH, Rosen FS, Alper CA (1980) Genetics of complement. Curr Top Hematol 3:111–174PubMedGoogle Scholar
  340. 152.
    Raum D, Glass D, Carpenter CB, Schur PH, Alper CA (1979) Mapping of the structural gene of the second component of complement with respect to the human major histocompatibility complex. Am J Hum Genet 31:35–41PubMedGoogle Scholar
  341. 153.
    Reid KBM (1976) Complete amino acid sequences of the three collagen-like regions present in subcomponent of the first component of human complement. Biochem J 179:367–371Google Scholar
  342. 154.
    Reid KBM (1983) Proteins involved in the activation and control of the two pathways of human complement. Biochem Soc Trans 11:1–12PubMedGoogle Scholar
  343. 155.
    Reid KBM, Edmondson J (1984) Location of the binding site in subcomponent C1q for plasma fibronectin. Acta Path Microbiol Immunol. Sect C Suppl 284, 92:11–17Google Scholar
  344. 156.
    Reid KBM, Gagnon J, Frampton J (1982) Completion of the amino acid sequences of the A and B chains of subcomponent C1q of the first component of human complement. Biochem J 203:559–569PubMedGoogle Scholar
  345. 157.
    Reid KBM, Proter RR (1976) Subunit composition and structure of subcomponent C1q of the first component of human complement. Biochem J 155:19–23PubMedGoogle Scholar
  346. 158.
    Reid KBM, Porter RR (1981) The proteolytic activation system of complement. Annu Rev Biochem 50:433–464PubMedGoogle Scholar
  347. 159.
    Reid KBM, Solomon E (1977) Biosynthesis of the first component of complement by human fibroblasts. Biochem J 167:647–660PubMedGoogle Scholar
  348. 160.
    Reid KBM, Thompson RA (1983) Characterization of a non-functional form of C1q found in patients with a genetically linked deficiency of C1q activity. Mol Immunol 20:1117–1125PubMedGoogle Scholar
  349. 161.
    Roos MH, Atkinson JP, Shreffler DC (1978) Molecular characterization of the Ss and Slp (C4) proteins of the mouse H-2 complex: subunit composition, chain size polymorphism and an intracellular (pro-Ss) precursor. J Immunol 121:1106–1115PubMedGoogle Scholar
  350. 162.
    Roos MH, Demant P (1982) Murine complement factor B (BF): sexual dimorphism and H-2-linked polymorphism. Immunogenetics 15:23–30PubMedGoogle Scholar
  351. 163.
    Roos MH, Kornfeld S, Shreffler DC (1980) Characterization of the oligosaccharide units of the fourth component of complement (Ss protein) synthesized by murine macrophages. J Immunol 124:2860–2863PubMedGoogle Scholar
  352. 164.
    Roos MH, Mollenhauer E, Demant P, Rittner C (1982) A molecular basis for the two locus (C4A and C4B) of the fourth component of human complement (C4). Nature 298:854–856PubMedGoogle Scholar
  353. 165.
    Rosa PA, Shreffler DC (1983) Cultured hepatocytes from mouse strains expressing high and low levels of the fourth component of complement differ in rate of synthesis of the protein. Proc Natl Acad Sci USA 80:2332–2336PubMedGoogle Scholar
  354. 166.
    Rosenfeld SI, Ruddy S, Austen KF (1969) Structural polymorphism of the fourth component of human complement. J Clin Invest 48:2283–2292PubMedGoogle Scholar
  355. 167.
    Rother K, Rother U, Muller-Eberhard HJ, Nilsson UR (1966) Deficiency of the sixth component of complement in rabbits with an inherited complement defect. J Exp Med 124:773–785PubMedGoogle Scholar
  356. 168.
    Rother U, Thorbecke GJ, Stecher-Levin VJ, Hurlimann J, Robert K (1968) Formation of C6 by rabbit liver tissue in vitro. Immunology 14:649–655PubMedGoogle Scholar
  357. 169.
    Ruddy S, Colten HR (1974) Rheumatoid arthritis: biosynthesis of complement proteins by synovial tissues. N Engl J Med 290:1284–1288PubMedGoogle Scholar
  358. 170.
    Sackstein R, Colten HR (1984) Molecular regulation of MHC class III (CH and factor B) gene expression in mouse peritoneal macrophages. J Immunol 133:1618–1626PubMedGoogle Scholar
  359. 171.
    Schorlemmer HU (1981) The role of complement in the function of the monocyte-macro-phage system. Hematol Blood Transfus 27:59–71Google Scholar
  360. 172.
    Schreiber RD, Müller-Eberhard HJ (1974) Fourth component of human complement: description of a three polypeptide chain structure. J Exp Med 140:1324–1335PubMedGoogle Scholar
  361. 173.
    Senger DR, Hynes RO (1978) C3 component of complement secreted by established cell lines. Cell 15:375–384PubMedGoogle Scholar
  362. 174.
    Shreffler DC, Owen RD (1963) A serologically detected variant in mouse serum: inheritance and association with the histocompatibility-2 locus. Genetics 48:9–25PubMedGoogle Scholar
  363. 175.
    Sim RB, Porter RR, Reid KBM, Gigli I (1977) The structure and enzymic activities of the C1r and C1s subcomponents of C1, the first component of human serum complement. Biochem J 163: 219–227PubMedGoogle Scholar
  364. 176.
    Skok J, Solomon E, Reid KBM, Thompson RA (1981) Distinct genes for fibroblast and serum C1q. Nature 292:549–551PubMedGoogle Scholar
  365. 177.
    Stecher VJ, Thorbecke GJ (1967) Sites of synthesis of serum proteins. I. Serum proteins produced by macrophages in vitro. J Immunol 99:643–652PubMedGoogle Scholar
  366. 178.
    Stecher VJ, Thorbeeke GJ (1967) Sites of synthesis of serum proteins. III. Production of beta-1-C, beta-1-E and transferrin by primate and rodent cell lines. J Immunol 99:660–662PubMedGoogle Scholar
  367. 179.
    Strunk RC, Whitehead AS, Cole FS (1985) Pretranslational regulation of the synthesis of the third Component of complement in human mononuclear phagocytes by the lipid A portion of lipopolysaccharide. J Clin Invest 76:985–990PubMedGoogle Scholar
  368. 180.
    Strunk RC, Kunke KS, Giclas PC (1983) Human peripheral blood monocyte-derived macrophages produce haemolytically active C3 in vitro. Immunology 49:169–174PubMedGoogle Scholar
  369. 181.
    Strunk RC, Tashjian AH, Colten HR (1975) Complement biosynthesis in vitro by rat hepatoma cell strains. J Immunol 114:331–335PubMedGoogle Scholar
  370. 182.
    Sundsmo JS (1982) The leukocyte complement system. Fed Proc 41:3094–3098PubMedGoogle Scholar
  371. 183.
    Sundsmo JS, Gotze O (1981) Human monocyte spreading induced by factor Bb of the alternative pathway of complement activation. J Exp Med 154:763–777PubMedGoogle Scholar
  372. 184.
    Sundsmo JS, Selberg JK, Chin AC, Gotze O (1979) A possible role for complement in lymphocyte activation. In: Kaplan JG (ed) The molecular basis of immune cell function. Elsevier-North Holland, Amsterdam, pp 466–478Google Scholar
  373. 185.
    Tedesco F, Densen P, Villa MA, Petersen BH, Sirchia G (1983) Two types of dysfunctional eighth component of complement (C8) molecules in C8 deficiency in man. Reconstitution of normal C8 from the mixture of two abnormal C8 molecules. J Clin Invest 71:183–191PubMedGoogle Scholar
  374. 186.
    Tedesco F, Bardare M, Giovannetti AM, Sirchia G (1980) A familial dysfunction of the eighth component of complement (C8). Clin Immunol Immunopathol 16:180–191PubMedGoogle Scholar
  375. 187.
    Tenner AJ, Cooper NR (1980) Analysis of receptor-mediated C1q binding to human peripheral blood mononuclear cells. J Immunol 125:1658–1664PubMedGoogle Scholar
  376. 188.
    Tenner AJ, Cooper NR (1981) Identification of types of cells in human peripheral blood that bind C1q. J Immunol 126:1174–1179PubMedGoogle Scholar
  377. 189.
    Tenner AJ, Cooper NR (1982) Stimulation of a human polymorphonuclear leukocyte oxidative response by the C1q subunit of the first complement component. J Immunol 128:2547–2552PubMedGoogle Scholar
  378. 190.
    Thomas ML, Janatova J, Gray WR, Tack BF (1982) Third component of human complement: localization of the internal thiolester bond. Proc Natl Acad Sci USA 79:1054–1058PubMedGoogle Scholar
  379. 191.
    Thomspon RA, Haeney M, Reid KBM, Davies JG, White RHR, Cameron AH (1980) A genetic defect of the C1q complement subcomponent associated with (immune complex) nephritis. N Engl J Med 303:22–24Google Scholar
  380. 192.
    Thorbecke GJ, Hochwald GM, van Furth R, Müller-Eberhard HJ, Jacobson EB (1965) Problems in determining the sites of synthesis of complement components. In: Wolstenholme GEW, Knight J (eds) CIBA symposium: Complement. Churchill, London, pp 99–119Google Scholar
  381. 193.
    Tschopp J, Esser AF, Spira TJ, Müller-Eberhard HJ (1981) Occurrence of an incomplete C8 molecule in homozygous C8 deficiency in man J Exp Med 154:1599–1607PubMedGoogle Scholar
  382. 194.
    Whaley K (1980) Biosynthesis of the complement components and the regulatory proteins of the alternative complement pathway by human peripheral blood monocytes. J Exp Med 151:501–516PubMedGoogle Scholar
  383. 195.
    Whaley K, Lappin D, Hamilton AO (1983) Serum-treated antigen-antibody complexes inhibit the production of C2 and factor B by mononuclear phagocytes. Immunology 48:255–263PubMedGoogle Scholar
  384. 196.
    Whaley K, Lappin D, Barkas T (1981) C2 synthesis by human monocytes is modulated by a nicotinic cholinergic receptor. Nature 293:580–583PubMedGoogle Scholar
  385. 197.
    Whitehead AS, Goldberger G, Woods DW, Markham AF, Colten HR (1983) Use of a cDNA clone for the fourth component of human complement (C4) for analysis of a genetic deficiency of C4 in guinea pig. Proc Natl Acad Sci USA 80:5387–5391PubMedGoogle Scholar
  386. 198.
    Whitehead AS, Sim RB, Bodmer WF (1981) A monoclonal antibody against human complement component C3: the production of C3 by human cells in vitro. Eur J Immunol 11:140–146PubMedGoogle Scholar
  387. 199.
    Whitehead AS, Solomon E, Chambers S, Bodmer WF, Povey S, Fey G (1982) Assignment of the structural gene for the third component of human complement to chromosome 19. Proc Natl Acad Sci USA 79:5021–5025PubMedGoogle Scholar
  388. 200.
    Wiebauer K, Domdey H, Diggelman H, Fey G (1982) Isolation and analysis of genomic DNA clones encoding the third component of mouse complement. Proc Natl Acad Sci USA 79:7077–7081PubMedGoogle Scholar
  389. 201.
    Winkelstein JA, Cork LC, Griffin DE, Griffin JW, Adams RJ, Price DL (1981) Genetically determined deficiency of the third component of complement in the dog. Science 212:1169–1170PubMedGoogle Scholar
  390. 202.
    Winkelstein JA, Johnson JP, Swift AJ, Ferry F, Yolken R, Cork LC (1982) Genetically determined deficiency of the third component of complement in the dog: in vitro studies on the complement system and complement-mediated serum activities. J Immunol 129:2598–2602PubMedGoogle Scholar
  391. 203.
    Woo P, Lachmann PJ (1981) The detection and characterization of a membrane protein with factor B-like activity on human lymphoid cells. Immunology 44:629–639PubMedGoogle Scholar
  392. 204.
    Woods DW, Markham AP, Ricker AT, Goldberger G, Colten HR (1982) Isolation of cDNA clones for the human complement protein factor B, a class III major histocompatibility complex gene product. Proc Natl Acad Sci USA 79:5661–5665PubMedGoogle Scholar
  393. 205.
    Wyatt HV, Colten HR, Borsos T (1972) Production of the second (C2) and fourth (C4) components of guinea pig complement by single peritoneal exudate cells. Evidence that one cell may produce both components. J Immunol 108:1609–1614PubMedGoogle Scholar
  394. 206.
    Ziccardi RJ, Cooper NR (1976) Physicochemical and functional characterization of the C1r subunit of the first complement component. J Immunol 116:496–503PubMedGoogle Scholar
  395. 207.
    Ziccardi RJ, Cooper NR (1976) Activation of C1r by proteolytic cleavage. J Immunol 116:504–509PubMedGoogle Scholar
  396. 208.
    Zimmer B, Hartung HP, Scharfenberger G, Bitter-Suermann D, Hadding U (1982) Quantitative studies of the secretion of complement component C3 by resident, elicited, and activated macrophages. Comparison with C2, C4 and lysosomal enzyme release. Eur J Immunol 12:426–430PubMedGoogle Scholar
  397. 1.
    Alper CA, Johnson AM, Birtch AG, Moore FD (1969) Human C’3: evidence for the liver as the primary site of synthesis. Science 163:286PubMedGoogle Scholar
  398. 2.
    Alper CA, Raum D, Awdeh Z, Petersen BH, Taylor PD, Starzl TE (1980) Studies of hepatic synthesis in vivo of plasma protein including orosomucoid, transferrin, alpha-1-antitrypsin, C8 and factor B. Clin Immunol Immunopathol 16:84PubMedGoogle Scholar
  399. 3.
    Auerbach HS, Baker RD, Matthews WJ, Colten HR (1984) Molecular mechanism for feedback regulation of C4 biosynthesis in guinea pig peritoneal macrophages. J Exp Med 159:1750PubMedGoogle Scholar
  400. 4.
    Barnum SR, Volanakis JE (1985) In vitro biosynthesis of complement protein D by U 937 cells. J Immunol 134:1799PubMedGoogle Scholar
  401. 5.
    Barnum SR, Volanakis JE (1985) Biosynthesis of complement protein D by Hep G2 cells: a comparison of D produced by Hep G2 cells, U 937 cells and blood monocytes. Eur J Immunol 15:1148PubMedGoogle Scholar
  402. 6.
    Beatty DW, Davis AE, Cole FS, Einstein LP, Colten HR (1981) Biosynthesis of complement by human monocytes. Clin Immunol Immunopathol 18:334PubMedGoogle Scholar
  403. 7.
    Bentley CD, Bitter-Suermann D, Hadding U, Brade V (1976) In vitro synthesis of factor B of the alternative pathway of complement by mouse peritoneal macrophages. Eur J Immunol 6:393PubMedGoogle Scholar
  404. 8.
    Bentley CD, Hadding U, Bitter-Suermann D, Brade V (1977) Effect of in vivo stimulation of mice on the secretion of factor B of the alternative pathway by peritoneal macrophages. Eur J Immunol 7:188PubMedGoogle Scholar
  405. 9.
    Bentley CD, Fries W, Brade V (1978) Synthesis of factors D, B, and P of the alternative pathway of complement activation as well as C3 by guinea pig peritoneal macrophages in vitro. Immunology 35:971PubMedGoogle Scholar
  406. 10.
    Bentley CD, Zimmer B, Hadding U (1981) The macrophage as a source of complement components. In: Pick E (ed) Lymphokines, vol 4. Academic, New York, p 197Google Scholar
  407. 11.
    Brade V, Bentley CD (1980) Synthesis and release of complement components by macrophages. In: van Furth R (ed) Mononuclear phagocytes. Functional aspects. Nijhoff, The Hague, p 1385Google Scholar
  408. 12.
    Brade V, Kreuzpaintner G (1982) Functional active complement components secreted by guinea pig peritoneal macrophages. Immunobiology 161:315PubMedGoogle Scholar
  409. 13.
    Caroll MC, Campbell RD, Bentley DR, Porter RR (1984) A molecular map of the human histocompatibility complex class III region Unking complement genes C4, C2 and factor B. Nature 307:237Google Scholar
  410. 14.
    Cole FS, Schneeberger EE, Lichtenberg NA, Colten HR (1982) Complement biosynthesis in human breast milk macrophages and blood monocytes. Immunology 46:429PubMedGoogle Scholar
  411. 15.
    Cole FS, Matthews WJ, Rossing TH, Gash DJ, Lichtenberg NA, Pennington JE (1983) Complement biosynthesis by human bronchoalveolar macrophages. Clin Immunol Immunopathol 27:153PubMedGoogle Scholar
  412. 16.
    Cole FS, Auerbach HS, Goldberger G, Colten HR (1985) Tissue-specific pretranslational regulation of complement production in human mononuclear phagocytes. J Immunol 134:Z610Google Scholar
  413. 17.
    Cole FS, Strunk RC, Perlmutter DH, Colten HR (1985) Gamma interferon (IFN-γ) increases expression of class III complement genes C2 and factor B in human monocytes and in murine fibroblasts transfected with human C2 and factor B genes. Complement 2:18 (Abstract)Google Scholar
  414. 18.
    Colten H (1976) Biosynthesis of complement. Adv Immunol 22:67PubMedGoogle Scholar
  415. 19.
    Colten HR (1982) Biosynthesis of the MHC-linked complement proteins (C2, C4, and factor B) by mononuclear phagocytes. Mol Immunol 19:1279PubMedGoogle Scholar
  416. 20.
    Colten HR (1983) Molecular genetics of the major histocompatibility linked complement genes. Springer Semin Immunopathol 6:149PubMedGoogle Scholar
  417. 21.
    Colten HR (1983) The complement genes. Immunol Today 4:151Google Scholar
  418. 22.
    De Ceulaer C, Papazoglon S, Whaley K (1980) Increased biosynthesis of complement components by cultured monocytes, synovial fluid macrophages and synovial membrane cells from patients with rheumatoid arthritis. Immunology 41:37PubMedGoogle Scholar
  419. 23.
    Götze O, Bianco C, Cohn ZA (1979) The induction of macrophage spreading by factor B of the properdin system. J Exp Med 149:372PubMedGoogle Scholar
  420. 24.
    Halbwachs L, Lachmann PJ (1976) Factor B of the alternative complement pathway on human lymphocytes. Scand J Immunol 5:697PubMedGoogle Scholar
  421. 25.
    Hartung HP, Hadding U (1983) Synthesis of complement by macrophages and modulation of their functions through complement activation. Springer Semin Immunopathol 6:283PubMedGoogle Scholar
  422. 26.
    Kawamoto Y, Keda M, Ichikawa H, Miyama A (1979) Complement protein and macrophages. I. Quantitative estimation of factor B produced by mouse peritoneal macrophages. Microbiol. Immunol. 23:987PubMedGoogle Scholar
  423. 27.
    Kreuzpaintner G, Brade V (1983) Effect of cycloheximide and of anti-C3 Fab’ on the intrinsic synthesis and secretion of lysosomal enzyme and of complement components by guinea pig peritoneal macrophages. Immunology 49:337PubMedGoogle Scholar
  424. 28.
    Lappin D, Moseley HL, Whaley K (1980) Effect of histamine on monocyte complement production. II. Modulation of protein secretion, degradation and synthesis. Clin Exp Immunol 42:515PubMedGoogle Scholar
  425. 29.
    Lappin DF, Whaley K (1982) Prostaglandins and prostaglandin synthetase inhibitors regulate the synthesis of complement components by human monocytes. Clin Exp Immunol 49:623PubMedGoogle Scholar
  426. 30.
    Lappin D. Whaley K (1982) Cyclic AMP modulation of complement protein production. Int J Immunopharmacol 4:415PubMedGoogle Scholar
  427. 31.
    Matthews WJ, Goldberger G, Marino JT, Einstein LP, Gash DJ, Colten HR (1982) Complement proteins C2, C4, and factor B: effect of glycosylation on their secretion and catabolism. Biochem J 204:839PubMedGoogle Scholar
  428. 32.
    Miyama A, Kawamoto Y, Ichikawa H, Okamoto K, Hara S, Inoue T (1980) Complement proteins and macrophages. II. The secretion of factor B by lipopolysaccharide-stimulated màcrophages. Microbiol Immunol 24:1223PubMedGoogle Scholar
  429. 33.
    Miyama A, Kawamoto Y, Ichikawa H, Mariyana T, Okamato K, Tahakashi T (1981) Complement proteins and macrophages. III. The production of factor B by particle ingesting macrophages. Microbiol Immunol 25:195PubMedGoogle Scholar
  430. 34.
    Morris KM, Aden DP, Knowles BB, Colten HR (1982) Complement biosynthesis by the human hepatoma-derived cell line HepG2. J Clin Invest 70:906PubMedGoogle Scholar
  431. 35.
    Newell SL, Atkinson JP (1983) Biosynthesis of C4 by mouse peritoneal macrophages. II. Comparison of C4 synthesis by resident and elicited cell populations. J Immunol 130:834PubMedGoogle Scholar
  432. 36.
    Ooi YM, Ooi BS (1982) Biosynthesis of membrane factor B by mouse peritoneal macrophages. Nature 298:389PubMedGoogle Scholar
  433. 37.
    Osthar K, Dybkjaer E (1974) Complement components on the surface of normal lymphocytes. Scand J Haematol 13:24Google Scholar
  434. 38.
    Perlmutter DH, Colten HR, Grossberger D, Strominger J, Seidman JG, Chaplin DD (1985) Expression of complement proteins C2 and factor B in transfected L cells. J Clin Invest 76:1449PubMedGoogle Scholar
  435. 39.
    Perlmutter DH, Goldberger G, Dinarello CA, Mizel SB, Colten HR (1985) Regulation of factor B, not C2, by interleukin 1 (IL-1) in murine fibroblasts transfected with the corresponding human genes. Complement 2:62 (Abstract)Google Scholar
  436. 40.
    Ramadori G, Sipe JD, Dinarello CA, Mizel SB, Colten HR (1985) Pretranslational modulation of a acute phase hepatic protein synthesis by murine recombinant interleukin 1 (IL-1) and purified human IL1. J Exp Med 162:930PubMedGoogle Scholar
  437. 41.
    Robertson J, Caldwell JR, Castle JR, Waldman RH (1976) Evidence for the presence of components of the alternative (properdin) pathway of complement activation in respiratory secretions. J Immunol 117:900PubMedGoogle Scholar
  438. 42.
    Ruddy S, Colten HR (1974) Rheumatoid arthritis. Biosynthesis of complement proteins by synovial tissues. N Engl J Med 290:1284PubMedGoogle Scholar
  439. 43.
    Sackstein R, Colten HR (1984) Molecular regulation of MHC class III (C4 and factor B) gene expression in mouse peritoneal macrophages. J Immunol 133:1618PubMedGoogle Scholar
  440. 44.
    Sundsmo JS (1982) The leucocyte complement system. Fed Proc 41:3094PubMedGoogle Scholar
  441. 45.
    Sundsmo JS, Chin JR, Papin RA, Fair DS, Werb Z (1985) Factor B, the complement alternative pathway serine proteinase, is a major constitutive protein synthesized and secreted by resident and elicited mouse macrophages. J Exp Med 161:306PubMedGoogle Scholar
  442. 46.
    Whaley K (1980) Biosynthesis of the complement components and the regulatory proteins of the alternative complement pathway by human peripheral blood monocytes. J Exp Med 151:501PubMedGoogle Scholar
  443. 47.
    Whaley K, Lappin D, Hamilton AO (1983) Serum-treated antigen-antibody complexes inhibit the production of C2 and factor B by mononuclear phagocytes. Immunology 48:255PubMedGoogle Scholar
  444. 48.
    Woo P, Lachmann PJ (1981) The detection and characterization of a membrane protein with factor B-like activity on human lymphoid cells. Immunology 44:629PubMedGoogle Scholar
  445. 1.
    Agnello V, Gell J, Tye MJ (1983) Partial genetic deficiency of the C4 component of complement in discoid lupus erythematosus and urticaria/angioedema. J Am Acad Dermatol 9:894–897PubMedGoogle Scholar
  446. 2.
    Albert ED, Rittner C, Grosse-Wilde H, Netzel B, Scholz S (1975) Recombination frequency and linkage disequilibrium between HL-A and Bf. In: Kissmeyer-Nielsen F (ed) Histocompatibility testing 1975. Munksgaard, Copenhagen, pp 941–944Google Scholar
  447. 3.
    Allen FH (1974) Linkage of HL-A and GBG. Vox Sang 27:382–384PubMedGoogle Scholar
  448. 4.
    Alper CA (1976) Inherited structural polymorphism in human C2: evidence for genetic linkage between C2 and Bf. J Exp Med 144:1111–1115PubMedGoogle Scholar
  449. 5.
    Alper CA, Propp RP (1968) Genetic polymorphism of the third component of human complement (C3). J Clin Invest 47:2181–2191PubMedGoogle Scholar
  450. 6.
    Alper CA, Johnson AM (1969) Immunofixation electrophoresis: a technique for the study of protein polymorphism. Vox Sang 17:445–452PubMedGoogle Scholar
  451. 7.
    Alper CA, Robin NI, Refetoff S (1971) Genetic polymorphism in rhesus C3 and Gc globulin. J Immunol 107:96–98PubMedGoogle Scholar
  452. 8.
    Alper CA, Rosen FS (1971) Studies of a hypomorphic variant of human C3. J Clin Invest 50:324–326PubMedGoogle Scholar
  453. 9.
    Alper CA, Azen EA, Geserick G, Goedde HW, Rittner C, Teisberg P, Wieme R (1972) Statement on the polymorphism of the third component of complement in man (C3). Vox Sang 25:18–20Google Scholar
  454. 10.
    Alper CA, Boenisch T, Watson L (1972) Genetic polymorphism in human glycine-rich beta-glycoprotein. J Exp Med 135:68–80PubMedGoogle Scholar
  455. 11.
    Alper CA, Hobart MJ, Lachman PJ (1975) Polymorphism of the sixth component of complement. In: Arbuthnott JP, Beeley JA (eds) Isoelectric focusing. Butterworth, LondonGoogle Scholar
  456. 12.
    Alper CA, Awdeh ZL, Raum D, Yunis EJ (1982) Extended major histocompatibility complex haplotypes in man: role of alleles analogous to murine t mutants. Clin Immun Immunopathol 24:276–285Google Scholar
  457. 13.
    Alper CA, Marcus D, Raum D, Petersen BH (1983) Genetic polymorphism in C8 betachain. Evidence for two unlinked genetic loci for the eighth component of human complement (C8). J Clin Invest 72:1526–1531PubMedGoogle Scholar
  458. 14.
    Alper CA, Raum D, Karp S, Awdeh ZL, Yunis EJ (1983) Serum complement supergenes of the major histocompatibility complex in man (complotypes). Vox Sang 45:62–67PubMedGoogle Scholar
  459. 15.
    Arvilommi H (1974) Capacity of complement C3 phenotypes to bind on the mononuclear cells in man. Nature 251:740–741PubMedGoogle Scholar
  460. 16.
    Awdeh ZL, Raum D, Alper CA (1979) Genetic polymorphism of human complement C4 and detection of heterozygotes. Nature 282:205–207PubMedGoogle Scholar
  461. 17.
    Awdeh ZL, Alper CA (1980) Inherited polymorphism of human C4 as revealed by de-sialyzation. Immunobiology 158:35–41PubMedGoogle Scholar
  462. 18.
    Awdeh ZL, Alper CA (1980) Inherited structural polymorphism of the fourth component of human complement. Proc Natl Acad Sci USA 77:3576–3580PubMedGoogle Scholar
  463. 19.
    Awdeh ZL, Raum D, Yunis EJ, Alper CA (1983) Extended HLA/complement allele haplotypes: evidence for T/t-like complex in man. Proc Natl Acad Sci USA 80:259–264PubMedGoogle Scholar
  464. 20.
    Awdeh ZL, Raum D, Yunis EJ, Alper CA (1984) An extended major histocompatibility haplotype with duplicated C4A. Immunobiology 164:317 (Abstract)Google Scholar
  465. 21.
    Azen EA, Smithies O (1968) Genetic polymorphism of C3 (beta-1c-globulin) in human serum. Science 162:905–907PubMedGoogle Scholar
  466. 22.
    Barbosa J, Weitkamp L, Guttormsen S, Johnson J, Szalapski E Jr (1979) Bf in early-onset insulin-dependent diabetes. Lancet 2:1239–1240PubMedGoogle Scholar
  467. 23.
    Baur MP, Danilovs JA (1980) Reference tables of two and three-locus haplotype frequencies for HLA-A, B, C, DR, BF, and GLO. In: Terasaki PI (ed) Histocompatibility testing 1980. UCLA Tissue Typing Laboratory Los Angeles, California, pp 1294–1310Google Scholar
  468. 24.
    Baur MP, Bertrams J, Louton TK, Mauff G, Rittner C, Schaede C (1984) Human C4 haplotype frequencies and their linkage disequilibria in the German population, (unpublished)Google Scholar
  469. 25.
    Belt KT, Carroll MC, Porter RR (1984) The structural basis of the multiple forms of human complement component C4. Cell 36:907–914PubMedGoogle Scholar
  470. 26.
    Belt KT, Yu YC, Carroll MC, Porter RR (1985) Polymorphism of human complement component C4. Immunogenetics 21:173–180PubMedGoogle Scholar
  471. 27.
    Bentley DR, Porter RR (1984) Isolation of cDNA clones for human complement component C2. Proc Natl Acad Sci USA 80:1212–1215Google Scholar
  472. 28.
    Bentley DR, Campbell RD (1985) Primary structure of C2 and relationship to other components of the complement system. Complement 2:9 (Abstract)Google Scholar
  473. 29.
    Bentley DR, Campbell RD, Cross SJ (1985) DNA polymorphism of the C2 locus. Immunogenetics 22:377–390PubMedGoogle Scholar
  474. 30.
    Bertrams J, Opferkuch W, Grosse-Wilde H, Luboldt W, Schuppien W, Kuwert E (1976) C2 hypocomplementemia in multiple sclerosis. Lancet 2:1358–1359PubMedGoogle Scholar
  475. 31.
    Bertrams J, Opferkuch W, Grosse-Wilde H, Schuppien W, Luboldt W, Kuwert E (1978) HLA-Dw2 associated C2-hypocomplementemic in multiple sclerosis. In: Opferkuch W, Rother K, Schultz DR (eds) Clinical aspects of the complement system. Thieme, Stuttgart, pp 212–216Google Scholar
  476. 32.
    Bertrams J, Baur MP, Grüneklee D, Hintzen U (1980) Association of Bf F1 and haplotype HLA-B18, Bf F1 with insulin-dependent diabetes mellitus. Immunobiology 158:129–133PubMedGoogle Scholar
  477. 33.
    Bertrams J, Dewald G, Spitznas M, Rittner C (1980) HLA-A, B, C, DR, Bf, and C2 alleles in insulin-dependent diabetes mellitus with proliferative retinopathy. Immunobiology 158:113–118PubMedGoogle Scholar
  478. 34.
    Bertrams J, Hintzen U, Schlicht V, Schoeps S (1982) C4: another marker for type 1 diabetes. Lancet 1:42Google Scholar
  479. 35.
    Bertrams J, Hintzen U, Schlicht V, Schoeps S, Gries FA, Louton TK, Baur MP (1984) Gene and haplotype frequencies of the fourth component of complement in type 1 diabetics and normal. Immunobiology 166:335–344PubMedGoogle Scholar
  480. 36.
    Bertrams J, Mauff G (1985) Another family with a silent allele of properdin factor B polymorphism (BF*QO). Hum Genet 70:321–323PubMedGoogle Scholar
  481. 37.
    Bitter-Suermann D, Krönke M, Brade V, Hadding U (1977) Inherited polymorphism of guinea pig factor B and C4: evidence for genetic linkage between the C4 and Bf loci. J Immunol 118:1822–1826PubMedGoogle Scholar
  482. 38.
    Bodmer WF (1979) Gene clusters and the HLA-system. In: Porter R, O’Connor M (eds) Human genetics: possibilities and realities. Ciba Foundation Symposium 66 (new series). Excerpt Medica, Elsevier, Amsterdam, pp 205–229Google Scholar
  483. 39.
    Böttger EC, Hoffmann T, Hadding U, Bitter-Suermann D (1985) Influence of genetically inherited complement deficiencies on humoral immune response in guinea pigs. J Immunol 135:4100–4107PubMedGoogle Scholar
  484. 40.
    Brandt-Casadevall C, Dimo-Simonin N, Gugler HR (1983) A rare phenotype C3*FFO.8 encountered in three successive generations of one family. Z Rechtsmed 91:135–138PubMedGoogle Scholar
  485. 41.
    Brönnestam R (1973) Studies of the C3 polymorphism. Relationship between C3 phenotypes and rheumatoid arthritis. Hum Hered 23:206–213PubMedGoogle Scholar
  486. 42.
    Brönnestam R, Cedergren B (1973) Studies of the C3 polymorphism. Relationship between C3 phenotypes and antibody titres. Hum Hered 23:214–219PubMedGoogle Scholar
  487. 43.
    Bruun-Petersen G, Lamm LU, Juncker Sorensen I, Buskjaer L, Mortensen JP (1981) Family studies of complement C4 and HLA in man. Hum Genet 58:260–267PubMedGoogle Scholar
  488. 44.
    Bruun-Petersen G, Lamm LU, Jacobsen BK, Kristensen T (1982) Genetics of complement C4. Two homoduplication haplotypes C4SC4S and C4FC4F in a family. Hum Genet 61:36–38PubMedGoogle Scholar
  489. 45.
    Budowle B, Rosemann JM, Go RCP, Louv W, Barger BO, Acton RT (1983) Phenotypes of the fourth complement component (C4) in Black Americans from the Southeastern United States. J Immunogenet 10:199–204PubMedGoogle Scholar
  490. 46.
    Bulusu LK, Clark EA (1982) Genetic polymorphism of C3 in Papio and Macaca species. Biochem Genet 20:859–864PubMedGoogle Scholar
  491. 47.
    Campbell RD, Porter RR (1983) Molecular cloning and characterization of the gene coding for human complement protein factor B. Proc. Natl Acad Sci USA 80:4464–4468PubMedGoogle Scholar
  492. 48.
    Campbell RD, Morley BJ, Sargent CA, Janjua NJ (1985) Molecular basis for allelic variation at the factor B locus. Complement 2:14 (Abstract)Google Scholar
  493. 49.
    Carroll MC, Capra JD (1978) Studies on the murine Ss protein: demonstration that the Ss protein is functionally the fourth component of complement. Proc Natl Acad Sci USA 75:2424–2427PubMedGoogle Scholar
  494. 50.
    Carroll MC, Capra JD (1979) Studies on murine Ss protein: demonstration that S region encodes structural gene for fourth component of complement. Proc Natl Acad Sci USA 76:4641–4644PubMedGoogle Scholar
  495. 51.
    Carroll MC, Passmore HC, Capra JD (1980) Structural studies on the murine fourth component of complement (C4). IV. Demonstration that C4 and Slp are encoded by separate loci. J Immunol 124:1745–1749PubMedGoogle Scholar
  496. 52.
    Carroll MC, Porter RR (1983) Cloning of a human complement component C4 gene. Proc Natl Acad Sci USA 80:264–267PubMedGoogle Scholar
  497. 53.
    Carroll MC, Campbell RD, Bentley DR, Porter RR (1984) A molecular map of the human major histocompatibility complex class III region linking complement genes C4, C2 and factor B. Nature 307:237–241PubMedGoogle Scholar
  498. 54.
    Carroll MC, Belt KT, Palsdottir A, Yu Y (1985) Molecular genetics of the fourth component of human complement and steroid 21-hydroxylase. Immunol Rev 87:40–60Google Scholar
  499. 55.
    Carroll MC, Campbell RD, Porter RR (1985) Mapping of steroid 21-hydroxylase genes adjacent to the complement component C4 genes in HLA, the major histocompatibility complex in man. Proc Natl Acad Sci USA 82:521–525PubMedGoogle Scholar
  500. 56.
    Carroll MC, Palsdottir A, Belt KT, Porter RR (1985) Deletion of complement C4 and steroid 21-hydroxylase genes in the HLA class III region. EMBO J 4:2547–2552PubMedGoogle Scholar
  501. 57.
    Carroll MC, Schneider PM, Rumsby G (1987) Molecular genetics of human steroid 21- hydroxylase genes. In: Gallo RC, Della Porta G, Albertini A (eds) Monoclonals and DNA probes in diagnostic and preventive medicine. Raven, New York, pp 63–72Google Scholar
  502. 58.
    Chan AC, Atkinson JP (1985) Oligosaccharide structure of human C4. J Immunol 134:1790–1798PubMedGoogle Scholar
  503. 59.
    Chaplin DD, Woods DE, Whitehead AS, Goldberger G, Colten HR, Seidman JG (1983) Molecular map of the murine S region. Proc Natl Acad Sci USA 80:6947–6951PubMedGoogle Scholar
  504. 60.
    Chung LP, Bentley DR, Reid KBM (1985) Molecular cloning anc characterization of the cDNA coding for C4b-binding protein, a regulatory protein of the classical pathway of the human complement system. J Biochem 230:133–141Google Scholar
  505. 61.
    Cole FS, Whitehead AS, Auerbach HS, Lint T, Zeitz HJ, Kilbridge P, Colten HR (1985) The molecular basis for genetic deficiency of the second component of human complement. N Engl J Med 313:11–16PubMedGoogle Scholar
  506. 62.
    Colten HR (1984) Expression of the MHC class III genes. Philos Trans R Soc Lond [Biol] 306:355–366Google Scholar
  507. 63.
    Colten HR, Borsos T, Rapp HJ (1970) Isoelectric focusing of human and guinea pig C2: polymorphism of guinea pig C2. Immunology 18:467–472PubMedGoogle Scholar
  508. 64.
    Colten HR, Alper CA (1972) Hemolytic efficiencies of genetic variants of human C3. J Immunol 108:1184–1187PubMedGoogle Scholar
  509. 65.
    Cross SJ, Edwards JH, Bentley DR, Campbell RD (1985) DNA polymorphism of the C2 and factor B genes. Immunogenetics 21:39–48PubMedGoogle Scholar
  510. 66.
    Curman B, Östberg L, Sandberg L, Malmheden-Eriksson I, Stalenheim G, Rask L, Peterson PA (1975) H-2 linked Ss protein is C4 component of complement. Nature 25:243–244Google Scholar
  511. 67.
    Curry RA, Dierich MP, Pellegrino MA, Hoch JA (1976) Evidence for linkage between HLA antigens and receptors for complement components C3b and C3d in human-mouse hybrids. Immunogenetics 3:465–471Google Scholar
  512. 68.
    David V, Fauchet R, Phengsavath H, Guenet L, LeGall JY (1983) Properdin factor B (Bf) polymorphism: subtyping of SS phenotypes. Hum Genet 64:189–190PubMedGoogle Scholar
  513. 69.
    Dawkins RL, Christiansen FT, Kay PH, Garlepp M, McCluskey J, Hollingsworth PN, Zilko PJ (1983) Disease associations with complotypes, supratypes and haplotypes. Immunol Rev 70:1--22Google Scholar
  514. 70.
    Day NK, L’Esperance P, Good RA, Michael FA, Hansen JA, Dupont B, Jersild C (1975) Hereditary C2 deficiency: genetic studies and associations with the HLA system. J Exp Med 141:1464–1469PubMedGoogle Scholar
  515. 71.
    De Bruijn MHL, Fey GH (1985) Human complement component C3: cDNA coding sequence and derived primary structure. Proc Natl Acad Sci USA 82:708–712PubMedGoogle Scholar
  516. 72.
    Demant P, Capkova J, Hinzova E, Voracova B (1973) The role of the histocompatibility- 2-linked Ss-Slp region in the control of mouse complement. Proc Natl Acad Sci USA 70:863–864PubMedGoogle Scholar
  517. 73.
    De Mouzon A, Ohayon E, Ducos J, Hauptmann G (1979) Bf and C4 markers for insulin- dependent diabetes in Basques. Lancet 2:1364PubMedGoogle Scholar
  518. 74.
    Dewald G, Rittner C (1979) Polymorphism of the second component of human complement (C2): observation of the rare phenotype C2 2 ( = C2 B) and data on the localization of the C2 locus in the HLA region. Vox Sang 37:47–54PubMedGoogle Scholar
  519. 75.
    DiScipio RG, Gagnon J (1982) Characterization of human complement components C6 and C7. Mol Immunol 19:1425–1431PubMedGoogle Scholar
  520. 76.
    Dissing J, Lund J, Soerensen H (1972) C3 polymorphism in a group of old arteriosclerotic patients. Hum Hered 22:466–472PubMedGoogle Scholar
  521. 77.
    Dodds AW, Law SK, Porter RR (1985) The origin of the very variable haemolytic activities of the common human complement component C4 allotypes including C4-A6. EMBO J 4:2239–2244PubMedGoogle Scholar
  522. 78.
    Dornan J, Allan P, Noel EP, Sampson L, Farid NR (1981) Alleles of the second component of complement (C2) in insulin-dependent diabetes mellitus. Hum Hered 31:211–213PubMedGoogle Scholar
  523. 79.
    Dupont B, Oberfîeld SE, Smithwick EM, Lee TD, Levine LS (1977) Close genetic linkage between HLA and congenital adrenal hyperplasia (21-hydroxylase deficiency). Lancet 2:1309PubMedGoogle Scholar
  524. 80.
    Dupont B, Pollack MS, Levine LS, O’Neill GJ, Hawkins BR, New MI (1980) Congenital adrenal hyperplasia. In: Terasaki PI (ed) Histocompatibility testing 1980. UCLA Tissue Typing Laboratory, Los Angeles, CA, pp 693–706Google Scholar
  525. 81.
    Dyer PA, Klouda PT, Harris R, Malliek NT (1980) Properdin factor B alleles in patients with idiopathic membranous nephropathy. Tissue Antigens 15:505–507PubMedGoogle Scholar
  526. 82.
    Dyer PA, Davidson JA (1983) Allotypes of properdin factor B (BF) and lymphocytoxic antibody production. Immunobiology 164:234–235 (Abstract)Google Scholar
  527. 83.
    Dykes DD, Crawford MH, Polesky HF (1981) Genetic variants of properdin factor B (Bf) in Papio hamadryas baboons. Folia Primatol (Basel) 36:226–231PubMedGoogle Scholar
  528. 84.
    Dykman TR, Cole JL, Iida K, Atkinson JP (1983) Polymorphism of human erythrocyte C3b/C4b receptor. Proc Natl Acad Sci USA 80:1698–1702PubMedGoogle Scholar
  529. 85.
    Dykman TR, Cole JL, Iida K, Atkinson JP (1983) Structural heterogeneity of the C3b/C4b receptor (CR1) on human peripheral blood cells. J Exp Med 157:2160–2165PubMedGoogle Scholar
  530. 86.
    Dybnan TR, Hatch JA, Atkinson JP (1984) Polymorphism of the human C3b/C4b receptor: identification of a third allele and analysis of receptor phenotypes in families and patients with systemic lupus erythematosus. J Exp Med 159:691–703Google Scholar
  531. 87.
    Farhud DD, Walter H (1973) Polymorphism of C3 in German, Bulgarian, Iranian and Angolan population. Human Genet 17:161–164Google Scholar
  532. 88.
    Fearon DT (1983) The human C3b receptor. Springer Semin Immunopathol 6:159–172PubMedGoogle Scholar
  533. 89.
    Ferreira A, Nussenzweig V (1975) Genetic linkage between serum levels of the 4th component of complement and the H-2 complex. J Exp Med 141:513–517PubMedGoogle Scholar
  534. 90.
    Ferreira A, Takahashi M, Nussenzweig V (1977) Purification and characterization of mouse serum protein with specific binding affinity for C4 (Ss protein). J Exp Med 146:1001–1018PubMedGoogle Scholar
  535. 91.
    Ferreira A, Nussenzweig V, Gigli I (1978) Structural and functional differences between the H-2 controlled Ss and Slp proteins. J Exp Med 148:1186–1197PubMedGoogle Scholar
  536. 92.
    Ferreira A, David CS, Nussenzweig V (1980) The murine H-2.7 specificity is an antigenic determinant of C4d, a fragment of the fourth component of the complement system. J Exp Med 151:1424–1435PubMedGoogle Scholar
  537. 93.
    Ferreira A, Michaelson J, Nussenzweig V (1980) H-2-controlled polymorphism of the gamma-chain of Slp (Sex-limited protein). Immunogenetics 11:491–497PubMedGoogle Scholar
  538. 94.
    Ferreira A, Michaelson J, Nussenzweig V (1980) A polymorphism of the gamma-chain of mouse C4 controlled by the S region of the major histocompatibility complex. J Immunol 125:1178–1182PubMedGoogle Scholar
  539. 95.
    Ferreira A, Eichinger D (1981) A simplified two-dimensional electrophoretic technique. J Immunol Methods 43:291–299PubMedGoogle Scholar
  540. 96.
    Fey GH (1983) Structure and expression of the C3 gene. Springer Semin Immunopathol 6:119–147PubMedGoogle Scholar
  541. 97.
    Fielder AHL, Walport MJ, Batchelor JR, Rynes RI, Black CM, Dodi IA, Hugher GRV (1983) Family study of the major histocompatibility complex in patients with systemic lupus erythematosus: importance of null alleles of C4A and C4B in determining disease susceptibility. Br Med J 286:425–428Google Scholar
  542. 98.
    Fleischnick E, Awdeh ZL, Raum D, Granados J, Alosco SM, Crigler JF, Gerald PS, Giles CM, Yunis EJ, Alper CA (1983) Extended MHC haplotypes in 21-hydroxylase deficiency congenital adrenal hyperplasia: shared genotypes in unrelated patients. Lancet 1:153–156Google Scholar
  543. 99.
    Ford EB (1940) Polymorphism and taxonomy. In: Huxley J (ed) The new systematics. Clarendon, Oxford, pp 493–513Google Scholar
  544. 100.
    Friend R, Handwerger BS, Kim Y, Michael AF, Yunis EJ (1975) C2 deficiency in man. Genetic relationship to a mixed lymphocyte reaction determinant. Immunogenetics 2:569–576Google Scholar
  545. 101.
    Fu SM, Kunkel HG, Brusman HP, Allen FH, Fotino M (1974) Evidence for linkage between HL-A histocompatibility genes and those involved in the synthesis of the second component of complement. J Exp Med 140:1108–1111PubMedGoogle Scholar
  546. 102.
    Gelfand MC, Sachs DH, Lieberman R, Paul WE (1974) Ontogeny of B lymphocytes III. H-2 linkage of a gene controlling the rate of appearance of complement receptor lymphocytes. J Exp Med 139:1142–1148PubMedGoogle Scholar
  547. 103.
    Geseriek G, Rose M (1973) The discovery of a genetic polymorphism in a conversion product of C3 by starch gelelectrophoneis: the Pt system. Methodological, genetic and forensic aspects. Vox Sang 25:11 (Abstract)Google Scholar
  548. 104.
    Geserick G, Waltz H, Patzelt D, Rose M (1980) Polymorphism of C3 in rabbits. Immunobiology 158:25–29PubMedGoogle Scholar
  549. 105.
    Geserick G, Patzelt D, Schröder H (1983) Zum Nachweis von Subtypen des Bf-Systems: Verteilung in einer Stichprobe der Berliner Bevölkerung (DDR). Biomed Biochem Acta 42:581–585Google Scholar
  550. 106.
    Geserick G, Patzelt D, Schröder H, Nagai T (1983) Isoelectrofocusing in the study the Bf system: existence of two common serotypes of the BfF allele. Vox Sang 44:178–182PubMedGoogle Scholar
  551. 107.
    Gibson DJ, Glass D, Carpenter CB, Schur P (1976) Hereditary C2 deficiency: diagnosis and HLA gene complex associations. J Immunol 116:1065–1070PubMedGoogle Scholar
  552. 108.
    Giles CM (1980) Antigens in plasma. A seminar on antigens on blood cells and body fluids. 33rd annual meeting of Am Assoc Blood Banks, Washington, DC, 1980, pp 33–49Google Scholar
  553. 109.
    Giles CM (1984) A new genetic variant for Chido. Vox Sang 46:149–156PubMedGoogle Scholar
  554. 110.
    Giles CM (1984) Partial inhibition of anti-Rg and anti-Ch reagents. I. Assessment for Rg/ Ch typing by inhibition. Vox Sang 48:160–166Google Scholar
  555. 111.
    Giles CM (1984) Partial inhibition of anti-Rg and anti-Ch reagents. II. Demonstration of separable antibodies for different determinants. Vox Sang 48:167–173Google Scholar
  556. 112.
    Giles CM, Batchelor JR, Dodi IA, Fielder AHL, Rittner C, Mauff G, Bender K, Levene C, Schreuder I, Wells L (1984) HLA and C4 haplotypes associated with partial inhibition of anti-Rg and anti-Ch. Immunogenetics 21:305–317Google Scholar
  557. 113.
    Glass D, Raum D, Gibson D, Stillman JS, Schur PH (1976) Inherited deficiency of the second component of complement. J Clin Invest 58:853–861PubMedGoogle Scholar
  558. 114.
    Glass D, Raum D, Balavitch D, Kagan E, Rabson A, Schur PH, Alper CA (1978) Inherited deficiency of the sixth component of complement: a silent or null gene. J Immunol 120:538–541PubMedGoogle Scholar
  559. 115.
    Götze O, Müller-Eberhard HJ (1971) Isolation of the precursor and the active form of the C3 activator from human serum. J Immunol 107:313 (Abstract)Google Scholar
  560. 116.
    Goldman MB, Cohen C, Stronski K, Bangalore S, Goldman JN (1982) Genetic control of C6 polymorphism and C6 deficiency in rabbits. J Immunol 128:43–48PubMedGoogle Scholar
  561. 117.
    Granados J, Karp S, Giles CM, Raum D, Awdeh ZI, Balner H, Yunis EJ, Alper CA (1982) There are two loci for C4 in the chimpanzee. IV. International Workshop for the Genetics of Complement Boston, MA, June, 1982 (Abstract)Google Scholar
  562. 118.
    Greiner J, Weber FJ, Mauff G, Baur MP (1980) Genetic polymorphisms of properdin factor B (Bf), the second component (C2), and the fourth component (C4) of complement in leprosy patients and healthy controls from Thailand. Immunobiology 158:134–138PubMedGoogle Scholar
  563. 119.
    Grosse-Wilde H, Doxiadis G, Krumbacher K, Dekkers-Bijma A, Kolb HJ (1983) Polymorphism of the fourth complement component the dog and linkage to the DLA system. Immunogenetics 18:537–540PubMedGoogle Scholar
  564. 120.
    Gross-Bellard M, Oudet P, Chambon P (1973) Isolation of high molecular weight DNA from mammalian cells. Eur J Biochem 36:32–38PubMedGoogle Scholar
  565. 121.
    Hall JE Jr, Alper CA (1977) Genetic polyporphism of the sixth component of complement in the Rhesus monkey. J Immunol 119:253–255PubMedGoogle Scholar
  566. 122.
    Hardy DB, Barnicot NA, Alper CA (1975) Protein polymorphism in the Hanuman langur (Presbytus). Folia Primatol (Basel) 23:173–187Google Scholar
  567. 123.
    Hatch JA, Atkinson JP, Suarez BK, Dykman TR (1984) Non-linkage of the C3b/C4b receptor with HLA. J Immunol 132:2168–2169PubMedGoogle Scholar
  568. 124.
    Haupt H, Heide K (1965) Isolierung und Eigenschaften eines beta-2-Glykoproteins aus Humanserum. Clin Chim Acta 12:419–424PubMedGoogle Scholar
  569. 125.
    Hauptmann G (1974) Lupus erythemateux aigus et deficits hereditaires en complement. Ann Dermatol Syphil 101:479–496Google Scholar
  570. 126.
    Hauptmann G (1980) C4 deficiency in early-onset insulin-dependent diabetes: a hypothesis. Lancet 1:1034PubMedGoogle Scholar
  571. 127.
    Hauptmann G, Sasportes M, Tongio MM, Mayer S, Dausset J (1976) The localization of the Bf locus within the MHS region on chromosome No. 6. Tissue Antigens 7:52–54PubMedGoogle Scholar
  572. 128.
    Hauptmann G, Tongio MM, Grosse-Wilde H, Mayer S (1977) Linkage between C2 deficiency and the HLA-A10, B18, Dw2/BfS haplotype in a French family. Immunogenetics 4:557–565Google Scholar
  573. 129.
    Hauptmann G, Tongio MM, Klein J, Mayer S, Cinqualbre J, Jeanblanc B, Kieny R (1980) Change in serum properdin factor B following human orthoptic liver transplantation. Immunobiology 158:76–81PubMedGoogle Scholar
  574. 130.
    Hauptmann G, Goetz J, Uring-Lambert B, Grosshans E (1986) C4 deficiency. In: Rother K (ed) Hereditary and acquired complement deficiencies. Prog Allergy 39, pp 232–249Google Scholar
  575. 131.
    Heilman U, Eggertsen G, Lundwall A, Engström A, Sjöquist J (1984) Primary sequence differences between Chido and Rodgers variants of tryptic C4d of the human complement system. FEBS Lett 170:254–258Google Scholar
  576. 132.
    Hing SN, Giles CM, Fielder AHL, Batchelor JR (1986) HLA haplotypes with C4B5: evidence for further allelic heterogeneity. Immunogenetics 23:151–155PubMedGoogle Scholar
  577. 133.
    Hobart MJ, Lachmann PJ, Alper CA (1975) Polymorphism of human C6. In: Peeters H (ed) Protides of the biological fluids. Pergamon, Oxford, pp 575–580Google Scholar
  578. 134.
    Hobart MJ, Lachmann PJ (1976) Allotypes of complement components in man. Transplant Rev 32:26–42PubMedGoogle Scholar
  579. 135.
    Hobart MJ, Cook PJL, Lachmann PJ (1977) Linkage studies with C6. J Immunogenet 4:423–428PubMedGoogle Scholar
  580. 136.
    Hobart MJ, Joysey V, Lachmann PJ (1978) Inherited structural variation and linkage relationships of C7. J Immunogenet 5:157–163PubMedGoogle Scholar
  581. 137.
    Hobart MJ, Vazguedes MA, Lachmann PJ (1981) Polymorphism of human C5. Ann Hum Genet 45:1–4PubMedGoogle Scholar
  582. 138.
    Holers VM, Cole JL, Lublin DM, Seya T, Atkinson JP (1985) Human C3b- and C4b-regu- latory proteins: a new gene family. Immunol Today 6:188–192Google Scholar
  583. 139.
    Huang CM, Klein J (1979) Murine antigen H-2.7: its genetics, tissue expression, and strain distribution. Immunogenetics 9:233–243Google Scholar
  584. 140.
    Iida K, Mornaghi R, Nussenzweig V (1982) Complement receptor (CR1) deficiency in erythrocytes from patients with systemic lupus erythematosus. J Exp Med 155:1427–1438PubMedGoogle Scholar
  585. 141.
    Isenman DE, Young JR (1984) The molecular basis for the difference in immune hemolysis activity of the Chido and Rodgers isotypes of human complement C4. J Immunol 132:3019–3027PubMedGoogle Scholar
  586. 142.
    Isenman DE, Young JR (1986) Covalent binding properties of the C4A and C4B isotypes of the fourth component of human complement on several C1-bearing cell surfaces. J Immunol 136:2542–2550PubMedGoogle Scholar
  587. 143.
    Kaidoh T, Natsuume-Sakai S, Takahashi M (1981) Murine C4-binding protein: a rapid purification method by affinity chromatography. J Immunol 126:463–467PubMedGoogle Scholar
  588. 144.
    Kaidoh T, Natsuume-Sakai S, Takahashi M (1981) Murine binding protein of the fourth component of complement: structural polymorphism and its linkage to the major histocompatibility complex. Proc Natl Acad Sci USA 78:3794–3798PubMedGoogle Scholar
  589. 145.
    Karp DR, Atkinson JP, Shreffler DC (1982) Genetic variation in glycosylation of the fourth component of murine complement: association with hemolytic activity. J Biol Chem 257:7330–7335PubMedGoogle Scholar
  590. 146.
    Kay PH, Dawkins RL (1984) Genetic polymorphism of complement C4 in dog. Tissue Antigens 23:151–155PubMedGoogle Scholar
  591. 147.
    Kay PH, Dawkins RL, Penhale JW (1985) The molecular structure of different polymorphic forms of canine C3 and C4. Immunogenetics 21:313–319PubMedGoogle Scholar
  592. 148.
    Kirk RL, Serjeantson SW, Theophilos J, Zimmet P, Whitehouse S, Court JM (1979) Age relationship between insulin-dependent diabetes mellitus and rare alleles of properdin factor B. Lancet 2:537PubMedGoogle Scholar
  593. 149.
    Klickstein LB, Wong WW, Smith JA, Morton C, Fearon DT, Weis JH (1985) Identification of long homologous repeats in human CR1. Complement 2:44 (Abstract)Google Scholar
  594. 150.
    Kolb WP, Müller-Eberhard HJ (1976) The membrane attack mechanism of complement: the three polypeptide chain structure of the eighth component (C8). J Exp Med 143:1131–1139Google Scholar
  595. 151.
    Kristensen T, Wetsel RA, Tack BF (1986) Structural analysis of human complement protein H: 1. Homology with C4b binding protein beta-2-glycoprotein I, and the Ba fragment of BF. J Immunol 136:3407–3411PubMedGoogle Scholar
  596. 152.
    Krönke M, Hadding U, Geczy AF, Weck AL, Bitter-Suermann D (1977) Linkage of guinea pig Bf and C4 to GPLA. J Immunol 119:2016–2018PubMedGoogle Scholar
  597. 153.
    Kunstmann G, Mauff G (1980) Genetic polymorphism of rabbit C6. Immunobiology 158:30–33PubMedGoogle Scholar
  598. 154.
    Kunstmann G, Mauff G, Pulverer G (1980) C6 polymorphism and rare alleles in Western Germany. Immunobiology 158:55–59PubMedGoogle Scholar
  599. 155.
    Kurtz F, Juif JG, Hauptmann GR, Goetz J (1983) Insulin-dependent diabetes in children and fourth component of complement (C4). In: Chiumello G, Sperling M (eds) Recent progress in pediatric endocrinology. Raven, New YorkGoogle Scholar
  600. 156.
    Lachmann PJ, Hobart MJ, Aston WP (1974) Complement technology. In: Weir DM (ed) Handbook of experimental immunology. Blackwell, Oxford, pp 5.1–5.17Google Scholar
  601. 157.
    Lachmann PJ, Grennan D, Martin K, Demant P (1975) Identification of Ss protein as murine C4. Nature 258:242–243PubMedGoogle Scholar
  602. 158.
    Lachmann PJ, Hobart MJ, Woo P (1978) Combined genetic deficiency of C6 and C1 in man. Clin Exp Immunol 33:193–203PubMedGoogle Scholar
  603. 159.
    Lachmann PJ, Hobart MJ (1979) The genetics of the complement system. In: Porter R, O’Connor M (eds) Human genetics: possibilities and realities. Ciba Foundation Symposium 66 (new series). Excerta Medica, Elsevier, Amsterdam, pp 231–250Google Scholar
  604. 160.
    Law SKA, Dodds AW, Porter RR (1984) A comparison of the properties of two classes, C4A and C4B of the human complement component C4. EMBO J 3:1819–1823PubMedGoogle Scholar
  605. 161.
    Lesavre PH, Müller-Eberhard HJ (1978) Mechanism of action of factor D of the alternative complement pathway. J Ep Med 148:1498–1509Google Scholar
  606. 162.
    Levi-Strauss M, Tosi M, Steinmetz M, Klein J, Meo T (1985) Multiple duplications of complement C4 gene correlate with H-2 controlled testosterone-independent expression of its sex-limited isoform, C4-Slp. Proc Natl Acad Sci USA 82:1746PubMedGoogle Scholar
  607. 163.
    Lundwall AB, Wetsel RA, Kristensen T, Whitehead AS, Woods DE, Ogden RC, Colten HR, Tack BF (1985) Isolation and sequence analysis of a cDNA clone encoding the fifth component of complement. J Biol Chem (in press)Google Scholar
  608. 164.
    Lundwall A, Hellmann U, Eggertsen G, Sjöquist J (1982) Isolation of tryptic fragments of human C4 expressing Chido and Rodgers antigens. Mol Immunol 19:1655–1665PubMedGoogle Scholar
  609. 165.
    Lundwall A, Wetsel RA, Domdey H, Tack BF, Fey GH (1984) Structure of murine complement component C3. Nucleotide sequence of cloned complementary and genomic DNA coding for the beta-chain. J Biol Chem 259:13851PubMedGoogle Scholar
  610. 166.
    Mäkalä AL, Eerola E, Lehtonen OP, Ruuska P, Lantto R (1983) Erythrocyte C3b receptors in juvenile rheumatoid arthritis. N Engl J Med 309:673Google Scholar
  611. 167.
    Maniatis T, Fritsch E, Sambrook J (1982) Molecular cloning. A laboratory manual. Cold Spring Harbor, New YorkGoogle Scholar
  612. 168.
    Martin A, Lachmann PJ, Halbwachs L, Hobart MJ (1976) Haemolytic diffusion plate assays for factors B and D of the alternative pathway of complement activation. Immunochemistry 13:317–324PubMedGoogle Scholar
  613. 169.
    Martin W, Ziegler C (1981) Bf-Immunfixation auf Cellogel-Folien. Blut 42:23–26PubMedGoogle Scholar
  614. 170.
    Mauff G (1977) Untersuchungen zum Komplementsystem des Menschen. Genetik und Biochemie der dritten und vierten Komponente des Komplementsystems sowie des Properdinfaktor B. Dissertation, University of KölnGoogle Scholar
  615. 171.
    Mauff G (1985) The complement system: genetics and function. In: Kocielak J, Wiktor-Jedrezejczak W, Kotelba-Witkowska B, Traczyk Z (eds) Trends in hematology. Institute of Hematology, WarszawGoogle Scholar
  616. 172.
    Mauff G, Freis H, Potrafki BG, Hummel K, Pulverer G (1974) Zur Problematik der Bestimmung seltener Varianten des C3-Polymorphismus. Untersuchungen an zwei Sippen mit den Merkmalen F0.65 und F0.5. Hum Genet 22:211–220Google Scholar
  617. 173.
    Mauff G, Potrafki BG, Freis H, Pulverer G (1974) Vergleichende Untersuchungen zum Polymorphismus des Posttransferrins (Pt) und der dritten Komponente des Humankomplementes. Hum Genet 21:75–80Google Scholar
  618. 174.
    Mauff G, Hummel K, Pulverer G (1975) Properdin Factor B (glycine-rich beta-glycoprotein or C3 proactivator)-polymorphism: genetic and biochemical aspects, first application to paternity cases. Z Immun Forsch 150:327–338Google Scholar
  619. 175.
    Mauff G, Gauchel FD, Hitzeroth HW (1976) Polymorphism of properdin factor B in South African, Negroid, Indian and Colored populations. Hum Genet 33:319–322PubMedGoogle Scholar
  620. 176.
    Mauff G, Bender K, Fischer B (1978) Genetic polymorphism of the fourth component of human complement. Vox Sang 34:296–301PubMedGoogle Scholar
  621. 177.
    Mauff G, Hauptmann G, Hitzeroth HW, Gauch F, Scherz R (1978) The nomenclature of properdin factor B allotypes. Immunobiology 154:115–120PubMedGoogle Scholar
  622. 178.
    Mauff G, Adam R, Wachauf B, Hitzeroth HW, Miller C (1980) Serum concentration and functional efficiency of factor B alleles. Immunobiology 158:86–90PubMedGoogle Scholar
  623. 179.
    Mauff G, Alper CA, Hobart M, Kühnl P, Kunstmann G, Meo T, Olving JH, Rittner C (1980) Statement on the nomenclature of human C6 polymorphism. Immunobiology 158:139–144PubMedGoogle Scholar
  624. 180.
    Mauff G, Federmann G, Hauptmann G (1980) A hemolytically inactive gene product of properdin factor B. Immunobiology 158:96–100PubMedGoogle Scholar
  625. 181.
    Mauff G, Alper CA, Awdeh ZL, Batchelor JR, Bertrams J, Bruun-Petersen G, Dawkins RL, Demant P, Edwards JH, Grosse-Wilde H, Hauptmann G, Klouda P, Lamm L, Mollenhauer E, Nerl C, Olaisen B, O’Neill G, Rittner C, Roos MH, Skanes V, Teisberg P, Wells L (1983) Statement on the nomenclature of human C4 allotypes. Immunobiology 164:184–191PubMedGoogle Scholar
  626. 182.
    Mauff G, Steuer M, Weck M, Bender K (1983) The C4 beta-chain: evidence for a genetically determined polymorphism. Hum Genet 64:186–188PubMedGoogle Scholar
  627. 183.
    Mauff G, Bender K, Giles CM, Goldmann S, Opferkuch W, Wachauf B (1984) Human C4 polymorphism: pedigree analysis of qualitative, quantitative, and functional parameters create a basis for phenotype interpretations. Hum Genet 65:362–372PubMedGoogle Scholar
  628. 184.
    McCluskey J, Kay PH, Stuckey M, Christiansen FT, Dawkins RL, Wilson G (1983) MHC supratype predicting heterozygous 21-hydroxylase deficiency. Lancet 1:764–765PubMedGoogle Scholar
  629. 185.
    McCluskey J, McCann VJ, Kay PH, Zilko PJ, Christiansen FT, O’Neill GJ, Dawkins RL (1983) HLA and complement allotypes in Type 1 (insulin-dependent) diabetes. Diabetologia 24:162–165PubMedGoogle Scholar
  630. 186.
    McLean RH, Bryan RK, Winkelstein J (1983) Hypomorphic variant of C3 slow (C3 S), hypocomplementemia, and hematuria. Immunobiology 164:275 (Abstract)Google Scholar
  631. 187.
    McMahan MR (1981) Studies of the genetic polymorphism of the third component of complement (C3) in non-human primates. Am J Primatol 1:320 (Abstract)Google Scholar
  632. 188.
    Meier EMM (1984) Biochemische und Assoziations-Studien zum Polymorphismus der vierten Komponente des Humankomplements. Thesis, University of BonnGoogle Scholar
  633. 189.
    Meo T, Krasteff T, Shreffler DC (1975) Immunochemical characterization of murine H2- controlled Ss (serum substance) protein through identification of its human homologue as the fourth component of complement. Proc Natl Acad Sci USA 72:4536–4539PubMedGoogle Scholar
  634. 190.
    Meo T, Atkinson J, Bernoco M, Bernoco D, Ceppellini R (1976) Mapping of the HLA locus controlling C2 structural variants and linkage disequilibrium between alleles C*2 and Bw15. Eur J Immunol 6:916–919Google Scholar
  635. 191.
    Meo T, Atkinson JP, Bernoco M, Bernoco D, Ceppellini R (1977) Structural heterogeneity of C2 complement protein and its genetic variants in man: a new polymorphism of the HLA region. Proc Natl Acad Sci USA 74:1672–1675PubMedGoogle Scholar
  636. 192.
    Mevag B, Olaisen B, Teisberg P (1981) Electrophoretic polymorphism of human C4 is due to charge differences in the alpha-chain, presumably in the C4d fragment. Scand J Immunol 14:303–307PubMedGoogle Scholar
  637. 193.
    Mevag B, Olaisen B, Teisberg P, Smith J (1983) Two C4 loci in macaca monkeys. Immunobiology 164:276–277 (Abstract)Google Scholar
  638. 194.
    Mevag B, Rogde S, Olaisen B, Gedde-Dahl T, Teisberg P, Tedesco F (1983) Genetic polymorphism of complement component C8. Proceedings, 10th Congress of forensic haemogenetics, München, 11–15 Oct 1983, pp 361–365Google Scholar
  639. 195.
    Miyakawa X, Xamada A, Kosaka K, Tsuda F, Kosugi E, Mayumi M (1981) Defective im- muneadherence (C3b) receptor on erythrocytes from patients with systemic erythematosus. Lancet 2:493–497PubMedGoogle Scholar
  640. 196.
    Möller G (ed) (1983) HLA and disease susceptibility. Immunol Rev 70Google Scholar
  641. 197.
    Mole JE, Anderson JK, Davison EA, Woods DE (1984) Complete primary structure for the zymogen of human complement factor B. J Biol Chem 260:2108Google Scholar
  642. 198.
    Mollenhauer E, Schmidt RE, Heinrichs M, Rittner C (1984) Scleroderma: possible significance of silent alleles at the C4B locus. Arthritis Rheum 27:711–712PubMedGoogle Scholar
  643. 199.
    Monahan JB, Sodetz JM (1980) Binding of the eighth component of human complement to the soluble cytolytic complex is mediated by its beta subunit. J Biol Chem 255:10579–10582PubMedGoogle Scholar
  644. 200.
    Monahan JB, Sodetz JM (1981) Role of the beta subunit in interaction of the eighth component of human complement with the membranebound cytolytic complex. J Biol Chem 256:3258–3262PubMedGoogle Scholar
  645. 201.
    Morley BJ, Campbell RD (1984) Internal homologies of the Ba fragment from human complement component factor B, a class III MHC antigen. EMBO J 3:153–157PubMedGoogle Scholar
  646. 202.
    Mortensen JP, Buskjaer L, Lamm LU (1980) Studies on the C2-deflciency in man. Immunology 39:541–549PubMedGoogle Scholar
  647. 203.
    Mortensen JP, Lamm LU (1981) Quantitative differences between complement factor-B phenotypes. Immunology 24:505–511Google Scholar
  648. 204.
    Nakamura S, Ooue O, Abe K (1984) Genetic polymorphism of the seventh component of complement in Japanese population. Hum Genet 66:279–281PubMedGoogle Scholar
  649. 205.
    Nakamura S, Ooue O, Akiyama K, Abe K (1984) Genetic polymorphism of the complement C6 and the haplotype analysis between C6 and C7 in a Japanese population. Hum Genet 68:138–141PubMedGoogle Scholar
  650. 206.
    Nakamura S, Abe K (1985) Genetic polymorphism of human factor I (C3b inactivator). Hum Genet 71:45–48PubMedGoogle Scholar
  651. 207.
    Nakamura S, Ooue O, Abe K (1986) A new BF F subtype by polyacrylamide gel isoelectric focusing. Hum Genet (in press)Google Scholar
  652. 208.
    Nakamura S, Ooue O, Abe K (1986) Genetic polymorphism of human complement component C81 in the Japanese population. Hum Genet (in press)Google Scholar
  653. 209.
    Nakamura T, Sekizawa A, Fujii T, Katairi CH (1986) Cosegregation of the polymorphic C4 with the MHC in the frog, Xenopus laevis. Immunogenetics 23:181–186PubMedGoogle Scholar
  654. 210.
    Natsuume-Sakai S, Hayakawa JI, Takahashi M (1978) Genetic polymorphism of murine C3 controlled by a single co-dominant locus on chromosome 17. J Immunol 121:491–498PubMedGoogle Scholar
  655. 211.
    Natsuume-Sakai S, Moriwaki K, Amano S, Hayakaawa JI, Kaidoh T, Takahashi M (1979) Allotypes of C3 in laboratory and wild mouse distinguished by alloantisera. J Immunol 123:216–221PubMedGoogle Scholar
  656. 212.
    Natsuume-Sakai S, Kaidoh T, Nonaka M, Takahashi M (1980) Structural polymorphism of murine C4 and its linkage to H-2. J Immunol 124:2714–2720PubMedGoogle Scholar
  657. 213.
    Natsuume-Sakai S, Moriwaki K, Migita S, Sudo K, Suzuki K, Lu DY, Wang C, Takahashi M (1983) Structural polymorphism of murine factor B controlled by a locus closely linked to the H-2 complex and demonstration of multiple alleles. Immunogenetics 18:117–124PubMedGoogle Scholar
  658. 214.
    Nelson DS (1963) Immune adherence. Immunology 3:131–180Google Scholar
  659. 215.
    Nerl CW, Mayeux R, O’Neill GJ (1982) Complement C4 allotypes in Alzheimer’s disease. Lancet 2:1343PubMedGoogle Scholar
  660. 216.
    Nerl C, Grosse-Wilde H, Valet G (1977) Association of low C2 and C4 serum levels with the HLA-Dw2 allele in healthy individuals. J Exp Med 148:704–708Google Scholar
  661. 217.
    New MI (ed) (1985) Congenital adrenal hyperplasia. Ann NY Acad Sci 458Google Scholar
  662. 218.
    Newell SL, Shreffler DC, Atkinson JP (1982) Biosynthesis of C4 by mouse peritoneal macrophages. J Immunol 129:653–659PubMedGoogle Scholar
  663. 219.
    Newman SL, Vogler LB, Feigin RD, Johnston R (1978) Recurrent septicemia associated with congenital deficiency of C2 and partial defciency of factor B and the alternative complement pathway. N Engl J Med 299:290–292PubMedGoogle Scholar
  664. 220.
    Nishimukai H, Kitamura H, Sano Y, Tamaki Y (1985) C3 variants in Japanese. Hum Hered 35:69–72PubMedGoogle Scholar
  665. 221.
    Noel EP, Sampson L, Pepper BM, Farid NR (1980) Polymorphism of the second component of complement (C2) in Graves disease. Hum Hered 30:245–247PubMedGoogle Scholar
  666. 222.
    Nonaka M, Natsuume-Sakai S, Kaidoh I, Nonoka M, Takahashi M (1980) Characterization of allotypic determinants of murine complement C3. J Immunol 125:2025–2030PubMedGoogle Scholar
  667. 223.
    Nonaka M, Takahashi M, Natsuume-Sakai S, Nonaka M, Tanaka S, Shimizu A, Honjo T (1984) Isolation of cDNA clones specifying the fourth component of mouse complement and its isotype, sex-limited protein. Proc Natl Acad Sci USA 81:6822–6826PubMedGoogle Scholar
  668. 224.
    Nonaka M, Nakayama K, Yeul YD, Takahashi M (1986) Complete nucleotide and derived amino acid sequences of sex-limited protein (Slp), nonfunctional isotype of the fourth component of mouse complement (C4). J Immunol 136:2989–2993PubMedGoogle Scholar
  669. 225.
    Nordhagen R, Olaisen B, Teisberg P, Gedde-Dahl T Jr (1980) Association between the electrophoretically determined C4 M haplotype products and partial inhibition of anti-Ch(a). J Immunogenet 7:301–306PubMedGoogle Scholar
  670. 226.
    Nordhagen R, Olaisen B, Teisberg P, Gedde-Dahl T Jr, Thorsby E (1981) C4 haplotype products and partial inhibition of anti-Rodgers sera. J Immunogenet 8:4895–4912Google Scholar
  671. 227.
    Ochs HD, Rosenfeld SI, Thomas ED, Giblett ER, Alper CA, Dupont B, Schaller JG, Gilliland BC, Hansen JA, Wedgwood RJ (1977) Linkage between the gene (or genes) controlling synthesis of the fourth component of complement and the major histocompatibility complex. N Engl J Med 296:470–475PubMedGoogle Scholar
  672. 228.
    Ogata RT (1985) Structure and expression of murine fourth component of complement (C4) and sex-limited protein (Slp). Immunol Rev 87:101–122PubMedGoogle Scholar
  673. 229.
    Ogata RT, Shreffler DC, Sepich DS, Lilly SP (1983) cDNA clone spanning the alpha- gamma subunit junction in the precursor of the murine fourth complement component (C4). Proc Natl Acad Sci USA 80:5061–5065PubMedGoogle Scholar
  674. 230.
    Ogata RT, Sepich DS (1985) Murine sex-limited protein: complete cDNA sequence and comparison with murine fourth component of complement. J Immunol 135:4239–4244PubMedGoogle Scholar
  675. 231.
    Ohno S (1970) Evolution by gene duplication. Springer, Berlin Heidelberg New YorkGoogle Scholar
  676. 232.
    Olaisen B, Teisberg P, Jonassen R (1980) The C4 system: quantitative studies of different genotypes. Immunobiology 158:82–85PubMedGoogle Scholar
  677. 233.
    Olaisen B, Teisberg P, Konassen R, Gedde-Dahl T Jr, Moen T, Thorsby E (1981) Complement loci of the HLA complex. Studies on families with intra-HLA crossovers and haplotype associations. Hum Immunol 2:247–254PubMedGoogle Scholar
  678. 234.
    Olving JH, Olaisen B, Teisberg P, Gedde-Dahl T Jr, Thorsby E (1977) Non-linkage between C6 and chromosome 6 markers. Hum Genet 37:125–129PubMedGoogle Scholar
  679. 235.
    Olving JH, Teisberg P, Olaisen B (1980) Polymorphism of the sixth component of complement (C6) in Norwegian Lapps. Hum Hered 30:211–214PubMedGoogle Scholar
  680. 236.
    Opferkuch W, Rother K, Schultz DR (eds) (1978) Clinical aspects of the complement system. Thieme, StuttgartGoogle Scholar
  681. 237.
    O’Neill GJ, Yang SY, Dupont B (1978) Two HLA-linked loci controlling the fourth component of human complement. Proc Natl Acad Sci USA 75:5165–5169PubMedGoogle Scholar
  682. 238.
    O’Neill GJ, Yang SY, Tegoli J, Berger R, Dupont B (1978) Chido and Rodgers blood groups are distinct antigenic components of human complement C4. Nature 272:668–670Google Scholar
  683. 239.
    O’Neill GJ, Miniter P, Pollack MS, Dupont B (1980) Different HLA antigen association for the functionally active and inactive product of the complement C4F1 allele. Hum Immunol 1:23–30PubMedGoogle Scholar
  684. 240.
    O’Neill GJ, Dupont B, Pollack MS, Levine LS, New MI (1982) Complement C4 allotypes in congenital adrenal hyperplasia due to 21-hydroxylase deficiency: further evidence for different allelic variants at the 21-hydroxylase locus. Clin Immunol Immunopathol 23:312–322PubMedGoogle Scholar
  685. 241.
    O’Neill GJ, Nerl CW, Kay PH, Christiansen FT, McCluskey J, Dawkins RL (1982) Complement C4 is a marker for adult rheumatoid arthritis. Lancet 2:214PubMedGoogle Scholar
  686. 242.
    O’Neill GJ, Nerl C, Pollack MS (1983) Analysis of active and inactive complement C4 complotypes associated with subtypes of HLA-B17 in different racial groups. Am J Hum Genet 35:309–317PubMedGoogle Scholar
  687. 243.
    O’Neill GJ, Mueller-Eckhaardt G, Prel L, Schendel DJ (1984) C4 allotyping distinguishes HLA-B14.1 and B14.2 subtypes. Immunogenetics 19:335–341PubMedGoogle Scholar
  688. 244.
    Palsdottir A, Cross SJ, Edwards JH, Carroll MC (1983) Correlation between a restriction fragment length polymorphism and C4A6 protein. Nature 306:615–616PubMedGoogle Scholar
  689. 245.
    Papiha SS, Bernal JE, Roberts DF, Habeebullah CM, Mishra SC (1979) C3 polymorphism in some Indian populations. Hum Hered 29:193–196PubMedGoogle Scholar
  690. 246.
    Parker KL, Atkinson JP, Roos MH, Shreffler DC (1980) Genetic and structural characterization of H-2 controlled allelic forms of murine C4. Immunogenetics 11:55–63PubMedGoogle Scholar
  691. 247.
    Parker KL, Capra JD, Shreffler DC (1980) Partial amino acid sequences of the murine fourth component of complement (C4): demonstration of homology with human C4 and identification of the amino-terminal subunit in Pro-C4. Proc Natl Acad Sci USA 77:4275–4278PubMedGoogle Scholar
  692. 248.
    Parker KL, Carroll MC, Shreffler DC, Capra JD (1981) Identification of H-2 controlled structural variants of the murine Slp protein and demonstration of cis-regulation of its expression. J Immunol 126:995–997PubMedGoogle Scholar
  693. 249.
    Passmore HC, Shreffler DC (1970) A sex limited serum protein variant in the mouse. Inheritance and association with the H-2 region. Biochem Genet 4:351–365PubMedGoogle Scholar
  694. 250.
    Penalva da Silva F, Hoecker GF, Day NK, Vienne K, Rubinstein P (1978) Murine complement component 3: genetic variation and linkage to H-2. Proc Natl Acad Sci USA 75:963–965Google Scholar
  695. 251.
    Pericak-Vance MA, Elston RC, Spira TJ, Band J (1982) Segregation and linkage analysis of immunochemical C8 levels in a family with C8 beta-chain deficiency. Am J Hum Genet 34:190AGoogle Scholar
  696. 252.
    Perlmutter DH, Colten HR, Grossberger D, Strominger J, Seidman JG, Chaplin DD (1985) Expression of complement proteins C2 and factor B in transfected L-cells. J Clin Invest 76:1449–1454PubMedGoogle Scholar
  697. 253.
    Petersen BH, Graham JA, Brooks GF (1976) Human deficiency of the eighth component complement. The requirement of C8 for serum Neisseria gonorrhoeae bactericidal activity. J Clin Invest 57:283–290PubMedGoogle Scholar
  698. 254.
    Porter RR (1983) Complement polymorphism, the major histocompatibility complex and associated diseases: a speculation. Mol Biol Med 1:161–168PubMedGoogle Scholar
  699. 255.
    Porter RR, Reid KBM (1979) Activation of the complement system by antibody-antigen complexes: the classical pathway. Adv Protein Chem 33:1–64PubMedGoogle Scholar
  700. 256.
    Prentice HL, Schneider PM, Strominger JL (1986) C4B gene polymorphism detected in a human cosmid clone. Immunogenetics 23:274–276PubMedGoogle Scholar
  701. 257.
    Raanford PR, Kirk RL, Zimmet P (1982) Distribution of complement factors BF, C2 and C6 in the Western Pacific. Acta Anthropogenet 6:23–32Google Scholar
  702. 258.
    Rao AG, Howard OMZ, Ng SC, Snyder JV, Whitehead AS, Colten HR, Sodetz JM (1986) Characterization of a cDNA clone encoding the alpha-subunit of the eighth component of human complement. Proceedings 6th International Congress of Immunology, Toronto, Canada, 6–11 July 1986, p 197 (Abstract)Google Scholar
  703. 259.
    Rapp HJ, Borsos T (1970) Molecular basis of complement action. Appleton-Century-Crofts, New YorkGoogle Scholar
  704. 260.
    Raum D, Glass D, Agnello V, Schur P, Alper CA (1978) Congenital deficiency of C2 and factor B. N Engl J Med 299:1313PubMedGoogle Scholar
  705. 261.
    Raum D, Alper CA, Stein R, Gabbay KH (1979) Genetic marker for insulin-dependent diabetes mellitus. Lancet 2:1208–1210Google Scholar
  706. 262.
    Raum D, Spencer MA, Balavitch D, Tidoman S, Merritt AD, Taggart RT, Petersen BH, Day NK, Alper CA (1979) Genetic control of the eighth component of complement. J Clin Invest 64:858–865PubMedGoogle Scholar
  707. 263.
    Raum D, Balner H, Petersen BH, Alper C (1980) Genetic polymorphism of serum complement components in the chimpanzee. Immunogenetics 10:455–468Google Scholar
  708. 264.
    Raum D, Donaldson VH, Rosen FS, Alper CA (1980) Genetics of complement. Curr Top Hematol 3:111–174PubMedGoogle Scholar
  709. 265.
    Raum D, Awdeh ZL, Alper CA (1981) BF types and the mode of inheritance of insulin- dependent diabetes mellitus (IDDM). Immunogenetics 12:59–74PubMedGoogle Scholar
  710. 266.
    Raum D, Awdeh Z, Anderson J, Strong L, Granados J, Teran L, Giblett E, Yunis EJ, Alper CA (1984) Human C4 haplotypes with duplicated C4A or C4B. Am J Hum Genet 36:72–79PubMedGoogle Scholar
  711. 267.
    Reid KBM (1985) Application of molecular cloning to studies on the complement system. Immunology 55:185–196PubMedGoogle Scholar
  712. 268.
    Reveille JD, Arnett FC, Wilson RW, Bias WB, McLean RH (1985) Null alleles of the fourth component of human complement and HLA haplotypes in familial systemic lupus erythematosus. Immunogenetics 21:299–311PubMedGoogle Scholar
  713. 269.
    Rigby PWJ, Dieckmann M, Rhodes C, Berg P (1977) Labelling deoxynucleic acid to high specific activity in vitro by nick translation with DNA polymerase I. J Mol Biol 113:237–251PubMedGoogle Scholar
  714. 270.
    Righetti PG (1983) Isoelectric focusing: theory, methodology and applications. In: Work TS, Burdon RH (eds) Laboratory techniques in biochemistry and molecular biology. Elsevier Biomedical, AmsterdamGoogle Scholar
  715. 271.
    Rittner C (ed) (1972) First international symposium and workshop on the polymorphism of the third component of the human complement system. Vox Sang 25:9–27Google Scholar
  716. 272.
    Rittner C (1983) Chromosomes and complement in scleroderma. Proceedings of the Scleroderma Symposium London, 12–13 May 1983, Smith, Kline and French Laboratories, London, pp 23–28Google Scholar
  717. 273.
    Rittner C, Rittner B (1973) Qualitative and quantitative comparison of C3 variant sera during the Bonn Workshop. Vox Sang 25:21–27PubMedGoogle Scholar
  718. 274.
    Rittner C, Rittner B (1974) Report 1973/1974 of the reference laboratory of the third component (C3) of the human complement system. Vox Sang 27:464–472PubMedGoogle Scholar
  719. 275.
    Rittner C, Grosse-Wilde H, Rittner B, Netzel B, Scholz S, Lorenz H, Albert ED (1975) Linkage group HL-A-MLC-BF (Properdin factor B). Hum Genet 27:173–183Google Scholar
  720. 276.
    Rittner C, Hauptmann G, Grosse-Wilde H, Grosshans E, Tongio MM, Mayer S (1975) Linkage between HL-A (major histocompatibility complex) and genes controlling the synthesis of the fourth component of complement. In: Kissmeyer-Nielsen F (ed) Histocompatibility testing 1975. Munksgard, Copenhagen, pp 945–954Google Scholar
  721. 277.
    Rittner C (1976) Genetic loci of components of the classical and alternate pathway of complement activation: a new dimension of the immunogenetic linkage group (HLA) on chromosome 6 in man. Hum Genet 35:1–20PubMedGoogle Scholar
  722. 278.
    Rittner C, Grosse-Wilde H, Albert ED (1976) Localization of the Bf locus within the HLA region. Report on a informative family and critical evaluation of available data of Bf mapping. Hum Genet 35:79–82PubMedGoogle Scholar
  723. 279.
    Rittner C, Opferkuch W, Wellek B, Grosse-Wilde H, Wernet P (1976) Lack of linkage between gene(s) controlling the synthesis of the seventh component of complement and the HLA region on chromosome No. 6 in man. Hum Genet 34:137–142PubMedGoogle Scholar
  724. 280.
    Rittner C, Dewald G, Berghoff E, Mollenhauer E (1979) C6-Polymorphismus der sechsten Komplementkomponente. Ein neues, aussagekräftiges System in der Abstammungsbegutachtung. Z Rechtsmed 83:17–25PubMedGoogle Scholar
  725. 281.
    Rittner C, Zaschke S, Berghoff E, Mollenhauer E, Opferkuch W, Baur MP (1980) Comparative studies of human C4 phenotypes their population genetics and association with HLA-B antigens. Immunobiology 158:119–128PubMedGoogle Scholar
  726. 282.
    Rittner C, Bertrams J (1981) On the significance of C2, C4, and Factor B polymorphisms in disease. Hum Genet 56:235–247PubMedGoogle Scholar
  727. 283.
    Rittner C, DeMarchi M, Mollenhauer E, Carbonara A (1984) Coeliac disease and C4A* QO: an association secondary to HLA-DR3. Tissue Antigens 23:130–134PubMedGoogle Scholar
  728. 284.
    Rittner C, Giles CM, Roos MH, Demant P, Mollenhauer E (1984) Genetics of human C4 polymorphism: detection and segregation of rare and duplicated haplotypes. Immunogenetics 19:321–333PubMedGoogle Scholar
  729. 285.
    Rittner C, Hargesheimer W, Mollenhauer E (1984) Population and formal genetics of the human C81 (alpha-gamma) polymorphism. Hum Genet 67:166–169PubMedGoogle Scholar
  730. 286.
    Rittner C, Kühnl P, Black CM, Pereira S, Welsh KI (1984) Scleroderma: possible association with the C4 system — a progress report. In: Albert ED, Baur MP, Mayr W (eds) Histocompatibility testing 1984. Springer, Berlin Heidelberg New YorkGoogle Scholar
  731. 287.
    Rittner C, Meier EMM, Stradmann B, Giles CM, Köchling R, Mollenhauer E, Kreth HW (1984) Partial C4 deficiency in subacute sclerosing panencephalitis. Immunogenetics 20:407–415PubMedGoogle Scholar
  732. 288.
    Rittner C, Tippett P, Giles CM, Mollenhauer E, Berger R, Nordhagen R, Buskjaer L, Bruun-Petersen G, Lamm L, Roos MH (1984) An international reference typing for Ch and Rg determinants on rare human C4 allotypes. Vox Sang 46:224–234PubMedGoogle Scholar
  733. 289.
    Rittner C, Hargesheimer W, Stradmann B, Bertrams J, Baur MP, Petersen BH (1986) Human C81 (alpha-gamma) polymorphism: detection in the alpha-gamma subunit on SDS-PAGE, formal genetics and linkage relationship. Am J Hum Genet 38:482–486PubMedGoogle Scholar
  734. 290.
    Robinson MA, Carroll MC, Johnson AH, Hartzman RJ, Belt KT, Kindt TJ (1985) Localization of C4 genes within the HLA complex by molecular genotyping. Immunogenetics 21:143–152PubMedGoogle Scholar
  735. 291.
    Rodriguez de Cordoba S, Dykman TR, Fellner FG, Ercilla G, Aqua M, Atkinson JP, Rubinstein P (1984) Evidence for linkage between the loci coding for the binding protein for the fourth component of human complement (C4BP) and for the C3b/C4b receptor. Proc Natl Acad Sci USA 81:7890–7892Google Scholar
  736. 292.
    Rodriguez de Cordoba S, Ferreira A, Nussenzweig V, Rubinstein P (1983) Genetic polymorphism of human C4-binding protein. J Immunol 131:1565PubMedGoogle Scholar
  737. 293.
    Rodriguez de Cordoba S, Rubinstein P (1984) Genetic polymorphism of human factor H (beta-1H). J Immunol 132:1906–1908Google Scholar
  738. 294.
    Rodriguez de Cordoba S, Rubinstein P, Ferreira A (1984) High resolution isoelectric focusing of immunoprecipitated proteins under denaturing conditions. A simple analytical method applied to the study of complement component polymorphisms. J Immunol Methods 60:165–172Google Scholar
  739. 295.
    Rodriguez de Cordoba S, Lublin DM, Rubinstein P, Atkinson JP (1985) Human genes for three complement components that regulate the activation of C3 are tightly linked. J Exp Med 161:1189–1195Google Scholar
  740. 296.
    Rogde S, Mevag B, Olaisen B, Gedde-Dahl T, Teisberg P (1984) Structural gene(s) for complement factor C8 (C8) on chromosome 1. Cytogenet Cell Genet 34:571 (Abstract)Google Scholar
  741. 297.
    Rogde S, Mevag B, Teisberg P, Gedde-Dahl T Jr, Tedesco F, Olaisen B (1985) Genetic polymorphism of complement component C8. Hum Genet 70:211–216PubMedGoogle Scholar
  742. 298.
    Roos MH, Atkinson JP, Shreffler DC (1978) Molecular characterization of the Ss and Slp (C4) proteins of the mouse H-2 complex: subunit composition, chain size polymorphism, and an intracellular (Pro-Ss) precursor. J Immunol 121:1106–1115PubMedGoogle Scholar
  743. 299.
    Roos MH, Demant P (1982) Murine complement factor B (BF): sexual dimorphism and H-2-linked polymorphism. Immunogenetics 15:23–30PubMedGoogle Scholar
  744. 300.
    Roos MH, Mollenhauer E, Demant P, Rittner C (1982) A molecular basis for the two locus model of human complement component C4. Nature 298:854–856PubMedGoogle Scholar
  745. 301.
    Roos MH, Demant P (1983) New polymorphisms of murine C4 are detected by high resolution electrophoresis. Immunobiology 164:289 (Abstract)Google Scholar
  746. 302.
    Roos MH, Giles CM, Demant P, Mollenhauer E, Rittner C (1984) Rodgers (Rg) and Chido (Ch) determinants on human C4: characterization of two C4 B5 subtypes one of which contains both Rg and Ch determinants. J Immunol 133:2634–2640PubMedGoogle Scholar
  747. 303.
    Rose M, Geserick G (1969) Ein neuer Serumpolymorphismus: Pt. Erste Hinweise für eine genetische Steuerung. Acta Biol Med Germ 23:351–356PubMedGoogle Scholar
  748. 304.
    Rosenfeld SI, Weitkamp LR, Ward F (1977) Hereditary C5 deficiency in man: genetic linkage studies. J Immunol 119:604–608PubMedGoogle Scholar
  749. 305.
    Rosenfeld SI, Weitkamp LR, Countryman JR (1978) Nonlinkage for a locus of human complement C5 deficiency to the complement C6 structural locus. Immunogenetics 7:95–97PubMedGoogle Scholar
  750. 306.
    Rosenfeld S, Ruddy S, Austen KF (1969) Structural polymorphism of the fourth component of human complement. J Clin Invest 48:2283–2292PubMedGoogle Scholar
  751. 307.
    Ross GD, Yount WJ, Walport MJ, Winfield JB, Parker CJ, Fuller CR, Taylor RP, Myones BL, Lachmann PJ (1985) Disease-associated loss of erythrocyte complement receptors (CR1, C3b receptors) in patients with systemic lupus erythematosus and other diseases involving autoantibodies and/or complement activation. J Immunol 135:2005–2014PubMedGoogle Scholar
  752. 308.
    Rother K, Rother U, Müller-Eberhard HJ, Nilsson UR (1966) Deficiency of the sixth component of complement in rabbits with an inherited complement defect. J Exp Med 12:773–777Google Scholar
  753. 309.
    Rother K, Rother U (eds) (1986) Complement deficiencies in animals and man. Prog Allergy 39. Karger, BaselGoogle Scholar
  754. 310.
    Rother U, Rother K (1961) Über einen angeborenen Komplementdefekt bei Kaninchen. Z Immun Forsch 121:224–228Google Scholar
  755. 311.
    Rubinstein P, Rosenfleld-Steele P, Day NK (1977) Murine factor B: electrophoretic polymorphism. Immunogenetics 5:363–366Google Scholar
  756. 312.
    Rubinstein P, Vienne K, Hoecker GF (1979) The location of the C3 and GLO (glyoxalase 1) loci of the IXth linkage group in mice. J Immunol 122:2584–2589PubMedGoogle Scholar
  757. 313.
    Sackstein R, Colten HR, Woods DE (1983) Phylogenetic conservation of a class III major histocompatibility complex antigen, factor B: isolation and nucleotide sequencing of mouse factor B cDNA clones. J Biol Chem 258:14693–14697PubMedGoogle Scholar
  758. 314.
    Sackstein R, Roos MH, Demant P, Colten HR (1984) Subdivision of the S region of the mouse major histocompatibility complex by identification of genomic polymorphisms of the class III genes. Immunogenetics 20:321PubMedGoogle Scholar
  759. 315.
    Scharfstein J, Ferreira A, Nussenzweig V (1978) Human C4 binding protein. I. Isolation and characterization. J Exp Med 148:207–211PubMedGoogle Scholar
  760. 316.
    Schifferli JA, Steiger G, Paccaud JP, Sjödholm AG, Hauptmann G (1986) Difference in the biological properties of the two forms of the fourth component of human complement (C4). Immunology 63:473–477Google Scholar
  761. 317.
    Schiotz PO, Hioby N, Norling N, Sörensen H (1978) C3 polymorphism in a Danish cystic fibrosis population and its possible association with antibody response. Hum Hered 28:293–300PubMedGoogle Scholar
  762. 318.
    Schneider PM, Rittner C, Carroll MC, Colten HR (1985) Genomic structure of the C4 and 21-OH loci in a family with a C4A (Ch+) gene product. Complement 2:71 (Abstract)Google Scholar
  763. 319.
    Schneider PM, Carroll MC, Alper CA, Rittner C, Whitehead AS, Yunis EJ, Colten HR (1986) Polymorphism of the human complement C4 and steroid 21-hydroxylase genes: restriction fragment length polymorphisms revealing structural deletions, homoduplications and size variants. J Clin Invest 78:650–657PubMedGoogle Scholar
  764. 320.
    Schröder R, Zander H, Andreas A, Mauff G (1983) Multiple sclerosis: immunogenetic analysis of sibpair doublecase families. II. Studies on the association of multiple sclerosis with C2, C4, BF, C3, C6, and Gm polymorphisms. Immunobiology 164:160–170PubMedGoogle Scholar
  765. 321.
    Sepich DS, Noonan DJ, Ogata RT (1985) Complete cDNA sequence of the fourth component of murine complement. Proc Natl Acad Sci USA 82:5895–5899PubMedGoogle Scholar
  766. 322.
    Shows TB, Alper CA, Bootsma D, Dorf M, Doublas T, Huisman T, Kits S, Klinger HP, Kozak C, Lalley PA, Lindsley D, McAlpine PJ, McDougall JK, Meera Khan P, Meisler M, Morton NE, Opitz JM, Partridge CW, Payne R, Roderick TH, Rubinstein P, Ruddle FH, Shaw M et al. (1979) International system for human gene nomenclature — ISGN (1979). Cell Genet 25:96–116Google Scholar
  767. 323.
    Shreffler DC (1976) The S region of the mouse major histocompatibility complex H-2: genetic variation and functional role in complement system. Transplant Rev 32:40–167Google Scholar
  768. 324.
    Shreffler DC, Owen RD (1963) A serologically detected variant in mouse serum: inheritance and association with the histocompatibility-2 locus. Genetics 48:9–25PubMedGoogle Scholar
  769. 325.
    Shreffler DC, Atkinson JP, Karp DR, Killion CC, Ogata RT, Rosa PA (1984) The C4 and Slp genes of the complement region of the murine H-2 major histocompatibility complex. Philos Trans R Soc Lond [Biol] 306:112–126Google Scholar
  770. 326.
    Southern EM (1975) Detection of specific sequences among DNA fragments separated by electrophoresis. J Mol Biol 98:503–517PubMedGoogle Scholar
  771. 327.
    Spinella DG, Passmore HC (1983) A newly defined murine alloantigen controlled by the S region of the H-2 complex: molecular association with the fourth component of complement. J Immunol 130:824–828PubMedGoogle Scholar
  772. 328.
    Steckel EW, York RG, Monahan JB, Sodetz J (1980) The eighth component of human complement: purification and physiochemical characterization of its unusual subunit structure. J Biol Chem 255:11997–12005PubMedGoogle Scholar
  773. 329.
    Suciu-Foca N, O’Neill GJ, Rubinstein P (1980) Evidence for the existence of a possible Bf “null” allele. In: Terasaki PI (ed) Histocompatibility testing 1980. UCLA Tissue Typing Laboratory Los Angeles, California, pp 935–936Google Scholar
  774. 330.
    Suzuki K, O’Neill GJ, Matsumoto H (1986) A product of the C4B locus lacking hemolytic activity. Hum Genet 73:101–103PubMedGoogle Scholar
  775. 331.
    Takahashi S, Takahashi M, Kaideh T, Natsuume-Sakai S, Takahashi T (1984) Genetic mapping of mouse C4-BP locus to the H-2D-Qa interval. J Immunol 132:6–8PubMedGoogle Scholar
  776. 332.
    Tappeiner G (1982) Disease states in genetic complement deficiencies. Int J Dermatol 21:175–188PubMedGoogle Scholar
  777. 333.
    Tedesco F (1986) C8 deficiency: clinical, biochemical and genetic aspects. Prog Allergy 39 pp 295–306PubMedGoogle Scholar
  778. 334.
    Tedesco F, Densen P, Villa MA, Petersen BH (1983) Two types of dysfunctional eighth component of complement (C8) molecules in C8 deficiency in man. J Clin Invest 71:183–191PubMedGoogle Scholar
  779. 335.
    Teisberg P (1970) High voltage agarose gel electrophoresis in the study of C3 polymorphism. Vox Sang 19:47–56PubMedGoogle Scholar
  780. 336.
    Teisberg P (1971) The distribution of C3 types in Norway. Hum Hered 21:154–161PubMedGoogle Scholar
  781. 337.
    Teisberg P, Olaisen B, Gedde-Dahl T Jr, Thorsby E (1975) On the localization of the Gb locus within the MHS region of chromosome No. 6. Tissue Antigens 5:257–261Google Scholar
  782. 338.
    Teisberg P, AkessonI, Olaisen B, Gedde-Dahl T Jr, Thorsby E (1976) Genetic polymorphism of C4 in man and localization of a structural C4 locus to the HLA gene complex of chromosome 6. Nature 264:253–254PubMedGoogle Scholar
  783. 339.
    Teisberg P, Olaisen B, Gedde-Dahl T Jr, Thorsby E, Nordhagen R (1980) Further investigations on the genetics of the C4 protein polymorphism. Immunobiology 158:45–49PubMedGoogle Scholar
  784. 340.
    Teisberg P, Olaisen B, Nordhagen R, Thorsby E, Gedde-Dahl T Jr (1980) A haemolytically non-active C4 gene product. Immunobiology 158:91–95PubMedGoogle Scholar
  785. 341.
    Teng YS, Tan SG (1982) Subtyping of properdin factor B (Bf) by isoelectrofocusing. Hum Hered 32:362–366PubMedGoogle Scholar
  786. 342.
    Tilley CA, Romans DG, Crookston MC (1978) Localization of Chido and Rodgers determinants to the C4d fragment of human C4. Nature 276:713–715PubMedGoogle Scholar
  787. 343.
    Tokunaga K, Araki C, Juji T, Omoto K (1981) Genetic polymorphism of the complement C2 in Japanese. Hum Genet 58:213–216PubMedGoogle Scholar
  788. 344.
    Tokunaga K, Araki C, Juji T, Omoto K (1982) Polymorphism of properdin factor B in Japanese. Description of a rare variant and data of association with HLA and C2. Hum Genet 60:42–45PubMedGoogle Scholar
  789. 345.
    Tokunaga K, Yukiyama Y, Omoto K (1983) Polymorphism of the complement component C6 in Japanese. J Immunogenet 10:419–424PubMedGoogle Scholar
  790. 346.
    Tokunaga K, Omoto K, Akiyama N, Ando H, Juji T (1984) A new variant (C2*BJ) of the C2 polymorphism. Transplant Proc (in press)Google Scholar
  791. 347.
    Tokunaga K, Omoto K, Fujii Y, Juji T (1984) Joint report: haplotype study on HLA-link-age genetic markers. Transplant Proc (in press)Google Scholar
  792. 348.
    Tokunaga K, Omoto K, Sakurai M, Saji H, Maruya E, Juji T (1984) A possible BF “silent” allele (BF*QO). Transplant Proc (in press)Google Scholar
  793. 349.
    Tokunaga K, Omoto K, Yukiyama Y, Sakurai M, Saji H, Maruya E (1984) Further study on a BF silent allele. Hum Genet 67:449–451PubMedGoogle Scholar
  794. 350.
    Tokunaga K, Omoto K, Akaza T, Akiyama N, Amemiya H, Naito S, Sasazuki T, Satoh H, Juji T (1986) Haplotype study on C4 polymorphism in Japanese. Associations with MHC alleles, complotypes, and HLA-complement haplotypes. Hum Genet (in press)Google Scholar
  795. 351.
    Tosi M, Levi-Strauss M, Georgatsou E, Amor M, Meo T (1985) Duplications of complement and non-complement genes of the H-2S region: evolutionary aspects of the C4 isotypes and molecular analysis of their expression variants. Immunol Rev 87:151–184PubMedGoogle Scholar
  796. 352.
    Towbin H, Staehelin T, Gordon J (1979) Electrophoretic transfer of proteins from polyacrylamide gels to nitrocellulose sheets: procedure and some applications. Proc Natl Acad Sci USA 76:4350–4354PubMedGoogle Scholar
  797. 353.
    Uring-Lambert B, Giles CM, Goetz J, Tongio MM, Mayer S, Hauptmann G (1984) C4 haplotypes with duplicated C4A or C4B: frequency and associations with BF, C2, and HLA-A, B, C, DR alleles with special reference to the duplication B1, 2. In: Albert E, Baur MP, Mayr W (eds) Histocompatibility testing 1984. Springer, Berlin Heidelberg New York Tokyo, pp 604–608Google Scholar
  798. 354.
    Wahl GM, Stern M, Stark GR (1979) Efficient transfer of large DNA fragments from agarose gels to diazobenzoloxymethyl-paper and rapid hybridization using dextran sulfate. Proc Natl Acad Sci USA 76:3683–3687PubMedGoogle Scholar
  799. 355.
    Wank R, Schendel DJ, O’Neill GJ, Riethmüller G, Held E, Feucht HE (1984) Rare variant of complement C4 is seen in high frequency in patients with primary glomerulonephritis. Lancet 1:872–873PubMedGoogle Scholar
  800. 356.
    Weidinger S, Schwarzfischer F, Cleve H (1979) Properdin factor B polymorphism. An indication for the existence of a Bf allele. Z Rechtsmed 83:259–264PubMedGoogle Scholar
  801. 357.
    Weidinger S, Schwarzfischer F, Burgemeister R, Cleve H (1984) Two new Bf S subtypes revealed by isoelectric focusing and immunoflxation. Hum Genet 68:90–92PubMedGoogle Scholar
  802. 358.
    Wetsel RA, D’Eustachio P, Strunk RC, Ogata RT, Tack BF (1985) Fifth complement component: complete cDNA sequence, chromosomal assignment, and deficiency studies. Complement 2:86 (Abstract)Google Scholar
  803. 359.
    White PC, New MI, Dupont B (1984) HLA-linked congenital adrenal hyperplasia results from a defective gene encoding a cytochrome P-450 specific for steroid 21-hydroxylation. Proc Natl Acad Sci USA 81:7505–7509PubMedGoogle Scholar
  804. 360.
    White PC, Grossberger D, Onufer BJ, Chaplin DD, New MI, Dupont B, Strominger JL (1985) Two genes encoding steroid 21-hydroxylase are located near the genes encoding the fourth component of complement in man. Proc Natl Acad Sci USA 82:1089–1093PubMedGoogle Scholar
  805. 361.
    White PC, New MI, Dupont B (1985) Adrenal 21-hydroxylase cytochrome P-450 genes within the MHC class III region. Immunol Rev 87:123–150PubMedGoogle Scholar
  806. 362.
    Whitehead AS, Solomon E, Chambers SP, Povey S, Bodmer WF (1982) Assignment of the gene for the third component of human complement (C3) to chromosome 19 using human-mouse somatic cell hybrids. Cytogenet Cell Genet 13:183–184Google Scholar
  807. 363.
    Whitehead AS, Solomon E, Chambers S, Bodmer WF, Povey S, Fey G (1982) Assignment of the structural gene for the third component of human complement to chromosome 19. Proc Natl Acad Sci USA 79:5021–5025PubMedGoogle Scholar
  808. 364.
    Whitehead AS, Goldberger G, Woods DE, Markham AF, Colten HR (1983) Use of a cDNA clone for the fourth component of human complement (C4) for analysis of genetic deficiency of C4 in guinea pig. Proc Natl Acad Sci USA 80:5387–5391PubMedGoogle Scholar
  809. 365.
    Whitehead AS, Woods DE, Fleischnick E, Chin JE, Yunis EJ, Katz AJ, Gerald PS, Alper CA, Colten HR (1984) DNA polymorphism of the C4 genes. A new marker for analysis of the major histocompatibility complex. N Engl J Med 310:88–91PubMedGoogle Scholar
  810. 366.
    Whitehead AS, Colten HR, Chang CC, Demars R (1985) Localization of the human MHC- linked complement genes between HLA-B and HLA-DR by using HLA mutant cell lines. J Immunol 134:641–643PubMedGoogle Scholar
  811. 367.
    Whitehead AS, Sackstein R (1985) Molecular biology of the human and mouse MHC class III genes: phylogenetic conservation, genetics and regulation of expression. Immunol Rev 87:185–208PubMedGoogle Scholar
  812. 368.
    Whitehead AS, Schneider PM, Awdeh ZL, Fleischnick E, Blumenthal M, Costello W, Gerald PS, Yunis EJ, Alper CA (1986) The distribution of C4 DNA variants in relation to MHC alleles and extented haplotypes. Hum Immunol (in press)Google Scholar
  813. 369.
    Whitehouse DB, Putt W (1983) Immunological detection of the sixth complement component (C6) following flat bed polyacrylamide gel isoelectric focusing and electrophoretic transfer to nitrocellulose filters. Ann Hum Genet 47:1–8PubMedGoogle Scholar
  814. 370.
    Wieme RJ, Demeulenaere L (1967) Genetically determined electrophoretic variant of the human complement component C3. Nature 214:1042–1043PubMedGoogle Scholar
  815. 371.
    Wilson JG, Wong WW, Schnur PH, Fearon DT (1982) Mode of inheritance of decreased C3b receptors on erythrocytes of patients with systemic lupus erythematosus. N Engl J Med 307:981–986PubMedGoogle Scholar
  816. 372.
    Wilson J, Murphy EE, Wong WW, Klickstein LB, Weis JH, Fearon DT (1985) Restriction fragment length polymorphism of the CR1 gene correlating with low number of CR1 on erythrocytes. Complement 2:87 (Abstract)Google Scholar
  817. 373.
    Wong WW, Wilson JG, Fearon DT (1983) Genetic regulation of a structural polymorphism of human C3b receptor. J Clin Invest 72:685–692PubMedGoogle Scholar
  818. 374.
    Wong WW, Klickstein LB, Smith JA, Weis JH, Fearon DT (1985) Identification of a partial cDNA clone for the human receptor for complement fragments C3b/C4b. Proc Natl Acad Sci USA 82:7711–7715PubMedGoogle Scholar
  819. 375.
    Woods DE, Markham AF, Ricker AT, Goldberger G, Colten HR (1982) Isolation of cDNA clones for the human complement protein factor B, a class III major histocompatibility complex gene product. Proc Natl Acad Sci USA 79:5661–5665PubMedGoogle Scholar
  820. 376.
    Woods DE, Edge MD, Colten HR (1984) Isolation of a cDNA clone for the human complement protein C2 and its use in the identification of a restriction fragment length polymorphism. J Clin Invest 74:634–638PubMedGoogle Scholar
  821. 377.
    Yu CY, Belt KT, Giles CM, Porter RR (1985) Chido antigenic determinants. Complement 2:89 (Abstract)Google Scholar
  822. 378.
    Zhao TM (1983) Genetic polymorphism of C3 and Bf in the Chinese population. Hum Hered 33:36–38Google Scholar
  823. 379.
    Ziegler JB, Alper CA, Balner H (1975) Properdin factor B and histocompatibility loci linked in the rhesus monkey. Nature 254:609–611PubMedGoogle Scholar
  824. 380.
    Ziegler JB, Watson L, Alper CA (1975) Genetic polymorphism of properdin factor B in the rhesus: evidence for single subunit structure in primates. J Immunol 114:1649–1653PubMedGoogle Scholar
  825. 1.
    Loos M (1982) The classical complement pathway: mechanism of activation of the first component by antigen-antibody complex. Prog Allergy 30:135–192PubMedGoogle Scholar
  826. 2.
    Ishizaka T, Ishizaka K, Borsos R, Rapp H (1966) C1 fixation of C1 by G and M but not by A antibody. J Immunol 97:716–726PubMedGoogle Scholar
  827. 3.
    Müller-Eberhard HJ, Kunkel HG (1961) Isolation of a thermolabile serum protein which precipitates γ-globulin aggregates and participates in immune hemolysis. Proc Soc Exp Biol Med 106:291–295PubMedGoogle Scholar
  828. 4.
    Augener W, Grey HM, Cooper NR, Müller-Eberhard HJ (1971) The reaction of monomeric and aggregated immunoglobulins with C1. Immunochemistry 8:1011–1020PubMedGoogle Scholar
  829. 5.
    Bubb MO, Conradie JD (1976) The importance of quaternary structure in the expression of the #-binding site of IgM. Immunology 31:893–902PubMedGoogle Scholar
  830. 6.
    Hurst MM, Volanakis JE, Hester RB, Stroud RM, Bennett JC (1974) The structural basis for binding of complement by immunoglobulin M. J Exp Med 140:1117–1121PubMedGoogle Scholar
  831. 7.
    Hurst MM, Volanakis JE, Stroud RM, Bennett JC (1975) CT fixation and classical complement pathway activation by a fragment of the CH4 domain of IgM. J Exp Med 142:1322–1326PubMedGoogle Scholar
  832. 8.
    Plaut AG, Cohen S, Tomasi TB (1972) Immunoglobulin M: fixation of human complement by the Fc fragment. Science 176:55–56PubMedGoogle Scholar
  833. 9.
    Sledge CR, Bing DH (1973) Binding properties of the human complement protein C1q. J Biol Chem 248:2818–2823PubMedGoogle Scholar
  834. 10.
    McKenzie MR, Creevy N, Heh M (1971) The interaction of human IgM and C1q. J Immunol 106:65–68Google Scholar
  835. 11.
    Hyslop NE, Dourmashkin RR, Green NM, Porter RR (1970) The fixation of complement and the activated first component (#) of complementary by complexes formed between antibody and divalent hapten. J Exp Med 131:783–802PubMedGoogle Scholar
  836. 12.
    Borsos T, Rapp HJ (1965) Complement fixation on cell surfaces by 19S and 7S antibodies. Science 150:505–506PubMedGoogle Scholar
  837. 13.
    Borsos T, Rapp HJ (1965) Hemolysis titration based on fixation of the activated first component of complement: evidence that one molecule of hemolysis suffices to sensitize an erythrocyte. J Immunol 95:559–566PubMedGoogle Scholar
  838. 14.
    Mayer MM (1961) Development of the one-hit theory of immune hemolysis. In: Heidelberger M, Plescia OJ (eds) Immunochemical approaches to problems in microbiology. Rutgers University Press, New Brunswick NJ, pp 268–279Google Scholar
  839. 15.
    Isliker H, Jocot-Guillermod H, Waldesbühl M, von Feilenberg R, Cerrotini JC (1967) Complement fixation by different IgG proportions and fragments. In: Miescher, Grabar (eds) Immunopathology V. pp 197–206Google Scholar
  840. 16.
    Kehoe JM, Fougereau M (1969) Immunoglobulin peptide with complement fixing activity. Nature 224:1212–1213PubMedGoogle Scholar
  841. 17.
    Arlaud GJ, Meyer CM, Colomb MG (1976) Use of an IgG fragment prepared with particulate plasmin to study the C1 binding and activation. FEBS Lett 66:132–136PubMedGoogle Scholar
  842. 18.
    Lee JP, Painter RH (1980) Complement binding properties of two peptides from the C2 region of human IgG 1. Mol Immunol 17:1155–1162PubMedGoogle Scholar
  843. 19.
    Allan R, Isliker H (1974) Studies on the complement-binding site of rabbit immunoglobulin G. II. The reaction of rabbit IgG and its fragments with C1q. Immunochemistry 11:243–248PubMedGoogle Scholar
  844. 20.
    Colten HR, Borsos T, Rapp HJ (1967) Efficiency of the first component of complement (C’1) in the hemolytic reaction. Science 158:1590–1529PubMedGoogle Scholar
  845. 21.
    Golan MD, Hitschold T, Loos M (1981) The reconstitution of human C1, the first complement component. Binding of C1r and C1s to C1q influences the C1q conformation. FEBS Lett 128:281–285PubMedGoogle Scholar
  846. 22.
    Circolo A, Borsos T (1982) Lysis of hapten-labeled cells by anti-hapten IgG and complement: effect of cell surface hapten density. J Immunol 128:1118–1121PubMedGoogle Scholar
  847. 23.
    Müller-Eberhard HG (1969) Complement. Ann Rev Biochem 38:389–414PubMedGoogle Scholar
  848. 24.
    Heinz HP, Burger R, Golan MD, Loos M (1984) Activation of the first component of complement, C1, by a monoclonal antibody recognizing the C chain of C1q. J Immunol 132:804–808 #PubMedGoogle Scholar
  849. 25.
    Schultz DR, Arnold PI, Wu MC, Lo TM, Volanakis JR, Loos M (1979) Isolation and partial characterization of a polysaccharide in ant venom (Pseudomyrmex sp.) that activates the classical complement pathway. Mol Immunol 16:253–264PubMedGoogle Scholar
  850. 26.
    Schultz DR, Loos M, Bub F, Arnold PI (1980) Differentiation of hemolytically active fluid-phase and cell-bound human C1q by an ant venom-derived polysaccharide. J Immunol 124:1251–1257PubMedGoogle Scholar
  851. 27.
    Golan MD, Burger R, Loos M (1982) Conformational changes in C1q after binding to immune complexes: detection of neoantigens with monoclonal antibodies. J Immunol 129:445–447PubMedGoogle Scholar
  852. 28.
    Loos M, Borsos T, Rapp HJ (1972) Activation of the first component of complement. Evidence for an internal activation step. J Immunol 108:683–688PubMedGoogle Scholar
  853. 29.
    Loos M, Borsos T, Rapp HJ (1973) The first component of complement in serum: evidence for a hitherto unrecognized factor in C1 necessary for internal activation. J Immunol 110:205–212PubMedGoogle Scholar
  854. 30.
    Dodds AW, Sim RB, Porter RP, Kerr MA (1978) Activation of the first component of human complement (C1) by antibody-antigen aggregates. Biochem J 175:383–390PubMedGoogle Scholar
  855. 31.
    Porter RR, Reid KBM (1979) Activation of the complement system by antibody-antigen complexes: the classical pathway. Adv Protein Chem 33:1–71PubMedGoogle Scholar
  856. 32.
    Ziccardi RJ (1983) The first component of human complement (C1): activation and control. Springer Semin Immunopathol 6:213–230PubMedGoogle Scholar
  857. 33.
    Loos M (1982) Antibody independent activation of C1, the first component of complement. Ann Immunol (Paris) 133C:165–179Google Scholar
  858. 1.
    Arroyave CM, Müller-Eberhard HJ (1973) Interactions between human C5, C6, and C7 and their functional significance in complement-dependent cytolysis. J Immunol 111:536–545PubMedGoogle Scholar
  859. 2.
    Bokisch VA, Dierich MP, Müller-Eberhard HJ (1975) Third component of complement (C3): structural properties in relation to functions. Proc Natl Acad Sci USA 72:1989–1993PubMedGoogle Scholar
  860. 3.
    Borsos T, Rapp HJ, Colten HR (1970) Immune hemolysis and the functional properties of the second (C2) and fourth (C4) components of complement. I. Functional differences among C4 sites on cell surfaces. J Immunol 105:1439–1446PubMedGoogle Scholar
  861. 4.
    Brown EJ, Ramsey J, Hammer CH, Frank MM (1983) Surface modulation of classical pathway activation: C2 and C3 convertase formation and regulation on sheep, guinea pig, and human erythrocytes. J Immunol 131:403–408PubMedGoogle Scholar
  862. 5.
    Burger R, Deubel U, Hadding U, Bitter-Suermann D (1982) Identification of functionally relevant determinants on the complement component C3 with monoclonal antibodies. J Immunol 129:2042–2050PubMedGoogle Scholar
  863. 6.
    Chakravarti DN, Müller-Eberhard HJ (1985) Purification of human C6 to homogeneity: lack of evidence of serine protease nature. Complement 2:16Google Scholar
  864. 7.
    Conrad DH, Carlo JR, Ruddy S (1978) Interaction of β1H globulin with cell-bound C3b: quantitative analysis of binding and influence of alternative pathway components on binding. J Exp Med 147:1792–1805PubMedGoogle Scholar
  865. 8.
    Cooper NR (1975) Enzymatic activity of the second component of complement. Biochemistry 14:4245–4250PubMedGoogle Scholar
  866. 9.
    Cooper NR, Müller-Eberhard HJ (1968) A comparison of methods for the molecular quantitation of the fourth component of human complement. Immunochemistry 5:155–169PubMedGoogle Scholar
  867. 10.
    Cooper NR, Müller-Eberhard HJ (1970) The reaction mechanism of human C5 in immune hemolysis. J Exp Med 132:775–793PubMedGoogle Scholar
  868. 11.
    Daha MR, Hazefoet HM, van Es LA, Cats A (1980) Stabilization of the classical pathway C3 convertase #, by a factor #, isolated from serum of patients with systemic lupus erythematosus. Immunology 40:417–424PubMedGoogle Scholar
  869. 12.
    Dessauer A, Rother U, Rother K (1984) Freeze-thaw activation of the complement attack phase: I Separation of two steps in the formation of the active # complex. Acta Microbiol Immunol Scand [C] Suppl 284, 92:75–81Google Scholar
  870. 13.
    DiScipio RG (1981 a) The binding of human complement proteins C5, factor B, β1H and properdin to complement fragment C3b on zymosan. Biochem J 199:485–496PubMedGoogle Scholar
  871. 14.
    DiScipio RG (1981 b) The conversion of human complement component C5 into fragment C5b by the alternative-pathway C5 convertase. Biochem J 199:497–504PubMedGoogle Scholar
  872. 15.
    DiScipio RG, Smith CA, Müller-Eberhard HJ, Hugli TE (1983) The activation of human complement component C5 by a fluid phase C5 convertase. J Biol Chem 258:10629–10636PubMedGoogle Scholar
  873. 16.
    Fearon DT, Austen KF (1975) Properdin: binding to C3b and stabilization of the C3b-dependent C3 convertase. J Exp Med 142:856–863PubMedGoogle Scholar
  874. 17.
    Fernandez HN, Hugli TE (1978) Primary structural analysis of the polypeptide portion of human C5a anaphylatoxin. J Biol Chem 253:6955–6964PubMedGoogle Scholar
  875. 18.
    Fishelson Z, Müller-Eberhard HJ (1982) C3 convertase of human complement: enhanced formation and stability of the enzyme generated with nickel instead of magnesium. J Immunol 129:2603–2607PubMedGoogle Scholar
  876. 19.
    Fishelson Z, Pangburn MK, Müller-Eberhard HJ (1983) C3 convertase of the alternative pathway. Demonstration of an active, stable C3b, Bb(Ni) complex. J Biol Chem 258:7411–7415PubMedGoogle Scholar
  877. 20.
    Gigli I, Austen KF (1969) Fluid phase destruction of C2hu by C1hu. I. Its enhancement and inhibition by homologous and heterologous C4. J Exp Med 129:679–696PubMedGoogle Scholar
  878. 21.
    Gigli I, Sorvillo J, Mecarelli-Halbwachs L, Leibowitch J (1981) Mechanism of action of the C4 nephritic factor. Deregulation of the classical pathway C3 convertase. J Exp Med 154:1–12PubMedGoogle Scholar
  879. 22.
    Goldlust MB, Shin HS, Hammer CH, Mayer MM (1974) Studies of complement complex C5b,6 eluted from EAC-6: reaction of C5b,6 with EAC4b,3b and evidence on the role of C2a and C3b in the activation of C5. J Immunol 113:998–1007PubMedGoogle Scholar
  880. 23.
    Hammer CH, Hänsch G, Gresham HD, Shin ML (1983) Activation of the fifth and sixth components of the human complement system: C6-dependent cleavage of C5 in acid and the formation of a biomolecular lytic complex, C5b,6a. J Immunol 131:892–898PubMedGoogle Scholar
  881. 24.
    Harrison RA, Thomas ML, Tack BF (1981) Sequence determination of the thiol ester site of the fourth component of human complement. Proc Natl Acad Sci USA 78:7388–7392PubMedGoogle Scholar
  882. 25.
    Isenman D, Kells DIC (1982) Conformational and functional changes in the fourth component of human complement produced by nucleophilic modification and by proteolyis with Cls. Biochemistry 21:1109–1117PubMedGoogle Scholar
  883. 26.
    Isenman DE, Young JR (1984) The molecular basis for the difference in immune hemolysis activity of the Chido and Rodgers isotypes of human complement component C4. J Immunol 132:3019–3027PubMedGoogle Scholar
  884. 27.
    Isenman DE, Podack ER, Cooper NR (1980) The interaction of C5 with C3b in free solution: a sufficient condition for cleavage by a fluid phase C3/C5 convertase. J Immunol 124:326–331PubMedGoogle Scholar
  885. 28.
    Isenman DE, Kells DIC, Cooper NR, Müller-Eberhard HJ; Pangburn MK (1981) Nucleophilic modification of human complement protein C3: correlation of conformational changes with acquisition of C3b-like functional properties. Biochemistry 20:4458–4467PubMedGoogle Scholar
  886. 29.
    Janatova J, Tack BF (1981) Fourth component of human complement: studies of an amine- sensitive site comprised of a thiol component. Biochemistry 20:2394–2402PubMedGoogle Scholar
  887. 30.
    Janatova J, Lorenz PE, Schechter AN, Prahl JW, Tack BF (1980) Third component of human complement: appearance of a sulfhydryl group following chemical or enzymic inactivation. Biochemistry 19:4471–4478PubMedGoogle Scholar
  888. 31.
    Kerr MA (1980) The human complement system: assembly of the classical pathway C3 convertase. Biochem J 189:173–181PubMedGoogle Scholar
  889. 32.
    Kitamura H, Matsumoto M, Nagaki K (1984) C3-independent immune haemolysis: haemolysis of EAC14oxy2 cells by C5-C9 without participation of C3. Immunology 53:575–582PubMedGoogle Scholar
  890. 33.
    Kolb WP, Kolb LM, Savary JR (1982) Biochemical characterization of the sixth component (C6) of human complement. Biochemistry 21:294–301PubMedGoogle Scholar
  891. 34.
    Lachmann PJ, Thompson RA (1970) Reactive lysis: the complement-mediated lysis of unsensitized cells. II. The characterization of activated reactor as # and the participation of C8 and C9. J Exp Med 131:643–657PubMedGoogle Scholar
  892. 34a.
    Law SKA, Dodds AW, Porter RR (1984) A comparison of the properties of two classes, C4A and C4B, of the human complement component C4. EMBO J 3:1819–1823PubMedGoogle Scholar
  893. 35.
    Law SK, Lichtenberg NA, Holcombe FH, Levine RP (1980 a) Interaction between the labile binding sites of the fourth (C4) and fifth (C5) human complement proteins and erythrocyte cell membranes. J Immunol 125:634–639PubMedGoogle Scholar
  894. 36.
    Law SK, Lichtenberg NA, Levine RP (1980 b) Covalent binding and hemolytic activity of complement proteins. Proc Natl Acad Sci USA 77:7194–7198PubMedGoogle Scholar
  895. 37.
    Lay WH, Nussenzweig V (1968) Receptors for complement on leukocytes. J Exp Med 128:991–1007PubMedGoogle Scholar
  896. 38.
    Mardiney MR, Müller-Eberhard HJ (1968) Ultrastructural localization of the third and fourth components of complement on complement-cell complexes. Am J Pathol 53:253–260PubMedGoogle Scholar
  897. 39.
    Müller-Eberhard HJ, Polley MA, Calcott MA (1967) Formation and functional significance of a molecular complex derived from the second and fourth components of human complement. J Exp Med 125:359–380PubMedGoogle Scholar
  898. 40.
    Nagasawa S, Stroud RM (1977) Cleavage of C2 by # into the antigenically distinct fragments C2a and C2b: demonstration of binding of C2b to C4b. Proc Natl Acad Sci USA 74:2998–3001PubMedGoogle Scholar
  899. 41.
    Nicholson-Weller A, Burge J, Fearon DT, Weller PF, Austen KF (1982) Isolation of a human erythrocyte membrane glycoprotein with decay-accelerating activity for C3 convertases of the complement system. J Immunol 129:184–189PubMedGoogle Scholar
  900. 42.
    Nilsson UR, Mandle RJ, McConnell-Mapes JA (1975) Human C3 and C5: subunit structure and modifications by trypsin and #. J Immunol 114:815–822PubMedGoogle Scholar
  901. 43.
    Okada H, Tanaka H, Okada N (1983) Prevention of complement activation on the homologous cell membrane of nucleated cells as well as erythrocytes. Eur J Immunol 13:340–344PubMedGoogle Scholar
  902. 44.
    Pangbum MK, Müller-Eberhardt HJ (1978) Complement C3 convertase: cell surface restriction of β1H control and generation of restriction on neuraminidase-treated cells. Proc Natl Acad Sci USA 75:2416–2420Google Scholar
  903. 45.
    Pangbum MK, Müller-Eberhard HJ (1980) Relation of a putative thioester bond in C3 to activation of the alternative pathway and the binding of C3b to biological targets of complement. J Exp Med 152:1102–1114Google Scholar
  904. 46.
    Polley MJ, Müller-Eberhard HJ (1967) Enhancement of the hemolytic activity of the second component of human complement by oxidation. J Exp Med 126:1013–1025PubMedGoogle Scholar
  905. 47.
    Rother U, Hänsch G, Rauterberg FW, Jungfer H, Rother K (1978) Deviated lysis: lysis of unsensitized cells by complement. V. Generation of the activity by low pH or low ionic strength. Z Immunitätsforsch 155:118–129Google Scholar
  906. 48.
    Schmitt M, Mussel HH, Dierich MP (1981) Qualitative and quantitative assessment of C3- receptor reactivities on lymphoid and phagocytic cells. J Immunol 126:2042–2047PubMedGoogle Scholar
  907. 49.
    Shin HS, Pickering RJ, Mayer MM (1971) The fifth component of the guinea pig complement system. III. Dissociation and transfer of C5b, and the probable site of C5b fixation. J Immunol 106:480–493PubMedGoogle Scholar
  908. 50.
    Sim E, Law SKA (1985) Hydrazine binds covalently to complement component C4. Different reactivity of C4A and C4B gene products. FEBS Lett 184:323–327PubMedGoogle Scholar
  909. 51.
    Sim RB, Twose TM, Paterson DS, Sim E (1981) The covalent-binding reaction of complement component C3. Biochem J 193:115–127PubMedGoogle Scholar
  910. 52.
    Sitomer G, Stroud RM, Mayer MM (1966) Reversible adsorption of C’2 by EAC’4: role of Mg2 +, enumeration of competent SAC’4, two-step nature of C’2 fixation and estimation of its efficiency. Immunochemistry 3:57–69PubMedGoogle Scholar
  911. 53.
    Strunk R, Colten HR (1974) The first component of human complement (C1): kinetics of reaction with its natural substrates. J Immunol 112:905–910PubMedGoogle Scholar
  912. 54.
    Tack BF, Morris SC, Prahl JW (1979) Third component of human complement: structural analysis of the polypeptide chains of C3 and C3b. Biochemistry 18:1497–150PubMedGoogle Scholar
  913. 355.
    Tack BF, Harrison RA, Janatova J, Thomas ML, Prahl JW (1980) Evidence for the presence of an internal thioester-bond in the third component of complement. Proc Natl Acad Sci USA 77:5764–5768PubMedGoogle Scholar
  914. 56.
    Tamerius JD, Pangburn MK, Müller-Eberhard HJ (1982) Selective inhibition of functional sites of cell-bound C3b hybridoma-derived antibodies. J Immunol 128:512–514PubMedGoogle Scholar
  915. 57.
    Vogt W (1979) Substrate modulation as a control mechanism of plasma multienzyme systems. In: Holzer H, Tschesche H (eds) Biological functions of proteinases. Springer, Berlin Heidelberg New York, pp 233–237Google Scholar
  916. 58.
    Vogt W, Dames W, Schmidt G, Dieminger L (1977) Complement activation by the properdin system: formation of a stoichiometric, C3 cleaving complex of properdin factor B with C3b. Immunochemistry 14:201–205PubMedGoogle Scholar
  917. 59.
    Vogt W, Schmidt G, von Buttlar B, Dieminger L (1978) A new function of the activated third component of complement: binding to C5, an essential step to C5 activation. Immunology 34:29–40PubMedGoogle Scholar
  918. 60.
    Vogt W, Hinsch B, Schmidt G, von Zabern I (1980) Cleavage of C5 by # in the fluid phase; support by high concentrations of C3. 4th International Congress of Immunology, Paris, 1980:15.1.24.Google Scholar
  919. 61.
    Vogt W, Hinsch B, Schmidt G, von Zabern I (1982) Function of the activated fourth component of complement (C4b) in activation of C2. FEBS Lett 144:195–198PubMedGoogle Scholar
  920. 62.
    Vogt W, Lühmann B, Hesse D (1984) “Inactivated” third component of complement (C3b-like C3; C3i) acquires C5 binding capacity and supports C5 activation upon covalent fixation to a solid surface. Complement 1:87–96PubMedGoogle Scholar
  921. 63.
    von Zabern I, Bloom EL, Chu V, Gigli I (1982 a) The fourth component of human complement treated with amines or chaotropes or frozen-thawed (C4b-like C4): interaction with C4 binding protein and cleavage by C3b/C4b inactivator. J Immunol 128:1433–1438Google Scholar
  922. 64.
    von Zabern I, Gigli I (1982 b) Conformational changes in complement C4 induced by activation, treatment with amines, chaotropes, or freezing-thawing, detectable by radioiodination using lactoperoxidase. J Immunol 128:1439–1442Google Scholar
  923. 65.
    von Zabern I, Hinsch B, Przyklenk H, Schmidt G, Vogt W (1980) Comparison of Naja n naja and Naja h haje cobra venom factors: correlation between binding affinity for the fifth component of complement and mediation of its cleavage. Immunobiology 157:499–514Google Scholar
  924. 66.
    von Zabern I, Nolte R, Vogt W (1981) Treatment of human complement components C4 and C3 with amines or chaotropic ions. Scand J Immunol 13:413–431Google Scholar
  925. 1.
    Götze O, Müller-Eberhard HJ (1976) The alternative pathway of complement activation. Adv Immunol 24:1–35PubMedGoogle Scholar
  926. 2.
    Pillemer L, Blum L, Lepow IH, Ross OA, Todd EW, Wardlaw AL (1954) The properdin system and immunity: I. Demonstration and isolation of a new serum protein, properdin, and its role in immune phenomena. Science 120:279–285PubMedGoogle Scholar
  927. 3.
    Nelson RA (1958) An alternative mechanism for the properdin system. J Exp Med 108:515–535PubMedGoogle Scholar
  928. 4.
    Pensky J, Hinz CF, Todd EW, Wedgwood RJ, Boyer JT, Lepow IH (1968) Properties of highly purified human properdin. J Immunol 100:142–258Google Scholar
  929. 5.
    Gewurz H, Shin HS, Mergenhagen SE (1968) Interactions of the complement system with endotoxic lipopolysaccharide: consumption of each of the six terminal complement components. J Exp Med 128:1049–1057PubMedGoogle Scholar
  930. 6.
    Schur PH, Becker EL (1963) Pepsin digestion of rabbit and sheep antibodies. The effect on complement fixation. J Exp Med 118:891–904PubMedGoogle Scholar
  931. 7.
    Sandberg AL, Osier AG, Shin HS, Oliveira B (1970) The biological activities of guinea pig antibodies: II. Modes of complement interaction with gamma1 and gamma2 immunoglobulins. J Immunol 104:329–334PubMedGoogle Scholar
  932. 8.
    Frank MM, May J, Gaither T, Ellman L (1971) In vitro studies of complement function in sera of C4-deficient guinea pigs. J Exp Med 134:176–187PubMedGoogle Scholar
  933. 9.
    Götze O, Müller-Eberhard HJ (1971) The C3-activator system: an alternate pathway of complement activation. J Exp Med 134:90s-108sPubMedGoogle Scholar
  934. 10.
    Boenisch T, Alper CA (1970) Isolation and properties of a glycine-rich β-glycoprotein of human serum. Biochim Biophys Acta 221:529–535PubMedGoogle Scholar
  935. 11.
    Haupt H, Heide K (1965) Isolierung und Eigenschaften eines β2-Glykoproteins aus Humanserum. Clin Chim Acta 12:419–424PubMedGoogle Scholar
  936. 12.
    Müller-Eberhard HJ, Götze O (1972) C3 proactivator convertase and its mode of action. J Exp Med 135:1003–1008PubMedGoogle Scholar
  937. 13.
    Ballow M (1977) Phylogenetics and ontogenetics of the complement systems. In: Day NK, Good RA (eds) Comprehensive immunology 2. Biological amplification systems in immunology. Plenum, New York, pp 183–204Google Scholar
  938. 15.
    Vogel C-W, Müller-Eberhard HJ (1982) The cobra venom factor-dependent C3 convertase of human complement. J Biol Chem 257:8292–829PubMedGoogle Scholar
  939. 916.
    Sjöholm AG, Braconier J-H, Söderström C (1982) Properdin deficiency in a family with fulminant meningococcal infections. Clin Exp Immunol 50:291–297PubMedGoogle Scholar
  940. 17.
    Sissons JGP, Schreiber RD, Perrin LH, Cooper NR, Müller-Eberhard HJ, Oldstone MBA (1979) Lysis of measles virus-infected cells by the purified cytolytic alternative complement pathway and antibody. J Exp Med 150:445–454Google Scholar
  941. 18.
    Schreiber RD, Götze O, Müller-Eberhard HJ (1976) Alternative pathway of complement: demonstration and characterization of initiating factor and its properdin-independent function. J Exp Med 144:1062–1075PubMedGoogle Scholar
  942. 19.
    Davis AE III, Ziegler JB, Gelfand EW, Rosen FS, Alper CA (1977) Heterogeneity of nephritic factor and its identification as an immunoglobulin. Proc Natl Acad Sci USA 74:3980–3983PubMedGoogle Scholar
  943. 20.
    Daha MR, Austen KF, Fearon DT (1978) Heterogeneity, polypeptide chain composition and antigenic reactivity of C3 nephritic factor. J Immunol 120:1389–1394PubMedGoogle Scholar
  944. 21.
    Lachmann PJ, Hughes-Jones NC (1984) Initiation of complement activation. Springer Semin Immunopathol 7:143–162PubMedGoogle Scholar
  945. 22.
    Lachmann PJ, Halbwachs L (1975) The influence of C3b inactivator (KAF) concentration on the ability of serum to support complement activation. Clin Exp Immunol 21:109–114PubMedGoogle Scholar
  946. 23.
    Pangburn MK, Schreiber RD, Müller-Eberhard HJ (1981) Formation of the initial C3 convertase of the alternative complement pathway. Acquisition of C3b-like activities by spontaneous hydrolysis of the putative thioester in native C3. J Exp Med 154:856–867PubMedGoogle Scholar
  947. 24.
    Tack BF, Harrison RA, Janatova J, Thomas ML, Prahl JW (1980) Evidence for presence of an internal thiolester bond in third component of human complement. Proc Natl Acad Sci USA 77:5764–5768PubMedGoogle Scholar
  948. 25.
    Müller-Eberhard HJ, Dalmasso AP, Calcott MA (1966) The reaction mechanism of β1C-globulin (C’3) in immune hemolysis. J Exp Med 123:33–54Google Scholar
  949. 26.
    Law SK, Levine RP (1977) Interaction between the third complement protein and cell surface macromolecules. Proc Natl Acad Sci USA 74:2701–2705PubMedGoogle Scholar
  950. 27.
    Law SK, Lichtenberg NA, Levine RP (1979) Evidence for an ester linkage between the labile binding site of C3b and receptive surfaces. J Immunol 123:1388–1394PubMedGoogle Scholar
  951. 28.
    Pangburn MK, Müller-Eberhard HJ (1980) Relation of a putative thioester bond in C3 to activation of the alternative pathway and the binding of C3b to biological targets of complement. J Exp Med 152:1102–1114PubMedGoogle Scholar
  952. 29.
    Janatova J, Lorenz PE, Schechter HN, Prahl JW, Tack BF (1980) Third component of complement: appearance of a sulfhydryl group following chemical or enzymatic activation. Biochemistry 19:4471–4478PubMed