Genetics of the Complement System

  • Chester A. Alper
  • Fred S. Rosen
Part of the Advances in Human Genetics book series (AHUG, volume 7)


The classic complement system consists of nine numbered components and two or three inhibitors. The first component comprises three subcomponents, Clq, Clr, and Cls, and it is the molecular interaction between Clq and aggregated IgG or IgM (as in an antigen-antibody complex) that initiates activation of the classic complement sequence (Fig. 1).


Complement System Hereditary Angioedema Arthus Reaction Immune Adherence Hereditary Deficiency 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Abramson, N., Alper, C. A., Lachmann, P. J., Rosen, F. S., and Jandl, J. H., 1971, Deficiency of C3 inactivator in man, J. Immunol. 107: 19.PubMedGoogle Scholar
  2. 2.
    Agarwal, D. P., Benkmann, H.-G., and Goedde, H. W., 1974. Levels of serum ß,C/ PIA globulin (C3) and its polymorphism in leprosy patients and healthy controls from Ethiopia and Mali, Humangenetik 21: 355.PubMedGoogle Scholar
  3. 3.
    Agarwal, D. P., Neméthy, I., Benkmann, H.-B., and Goedde, H. W., 1973, Genetic polymorphism of C3: levels of total hemolytic complement activity and /31e-globulin in different phenotypes of C3 in a North German population, Hum. Hered. 23: 357.PubMedGoogle Scholar
  4. 4.
    Agnello, V., deBracco, M. M. E., and Kunkel, H. G.. 1972, Hereditary C2 deficiency with some manifestations of systemic lupus erythematosus, J. Immunol. 108: 837.PubMedGoogle Scholar
  5. 5.
    Allen, F. H., Jr., 1974, Linkage of HL-A and GBG, Vox Sang. 27: 382.PubMedGoogle Scholar
  6. 6.
    Alper, C. A., 1970, Genetic polymorphism of human C3. in “Protides of the Biological Fluids,” Vol. 17 ( H. Peeters, ed.), pp. 295–300, Pergamon Press, Oxford.Google Scholar
  7. 7.
    Alper, C. A., and Jonhson, A. M., 1969, Immunofixation electrophoresis: a technique for the study of protein polymorphism, Vox Sang. 17: 445.PubMedGoogle Scholar
  8. 8.
    Alper, C. A., and Propp, R. P., 1967, An electrophoretic variant of human C’3 (Abstract), Clin. Res. 15: 291.Google Scholar
  9. 9.
    Alper, C. A., and Propp, R. P., 1968, Genetic polymorphism of the third component of human complement, J. Clin. Invest. 47: 2181.PubMedCentralPubMedGoogle Scholar
  10. 10.
    Alper, C. A., and Rosen, F. S., 1967, Studies of the in vivo behavior of human C’3 in normal subjects and patients, J. Clin. Invest. 46: 2021.PubMedCentralPubMedGoogle Scholar
  11. 11.
    Alper, C. A., and Rosen, F. S., 1971, Genetic aspects of the complement system, in “Advances in Immunology” (H. G. Kunkel and F. J. Dixon. eds.), pp. 251–290, Academic Press, New York.Google Scholar
  12. 12.
    Alper, C. A., and Rosen, F. S., 1971, Studies of a hypomorphic variant of human C3, J. Clin. Invest. 50: 324.PubMedCentralPubMedGoogle Scholar
  13. 13.
    Alper, C. A., Boenisch, T., and Watson, L., 1972, Genetic polymorphism in human glycine-rich beta-glycoprotein, J. Exp. Med. 135: 68.PubMedCentralPubMedGoogle Scholar
  14. 14.
    Alper, C. A., Goodkofsky, I., and Lepow, I. H.. 1973. The relationship of glycine-rich beta-glycoprotein to Factor B in the properdin system and to the cobra factor-binding protein of human serum, J. Exp. Med. 137: 424.PubMedCentralPubMedGoogle Scholar
  15. 15.
    Alper, C. A., Robin, N. I., and Refetoff, S.. 1971, Genetic polymorphism in rhesus C3 and Gc globulin, J. Immunol. 107: 96.PubMedGoogle Scholar
  16. 16.
    Alper, C. A., Rosen, F. S., and Lachmann, P. J., 1972, Inactivator of the third component of complement as an inhibitor in the properdin pathway, Proc. Natl. Acad. Sci. U.S.A. 69: 2910.PubMedCentralPubMedGoogle Scholar
  17. 17.
    Alper, C. A., Johnson, A. M., Birtch, A. G., and Moore, F. D., 1969, Human C’3: evidence for the liver as the primary site of synthesis, Science 163: 286.PubMedGoogle Scholar
  18. 18.
    Alper, C. A., Propp, R. P., Klemperer, M. R., and Rosen, F. S., 1969, Inherited deficiency of the third component of human complement (C’3). J. Clin. Invest. 48: 553.PubMedCentralPubMedGoogle Scholar
  19. 19.
    Alper. C. A., Abramson, N., Johnston, R. B., Jr., Jandl, J. H., and Rosen, F. S., 1970, Studies in vivo and in vitro on an abnormality in the metabolism of C3 in a patient with increased susceptibility to infection, J. Clin. Invest. 49: 1975.Google Scholar
  20. 20.
    Alper, C. A., Abramson, N., Johnston, R. B., Jr., Jandl, J. H., and Rosen, F. S., 1970, Increased susceptibility to infection associated with abnormalities of complement-mediated functions and of the third component of complement (C3), N. Engl. J. Med. 282: 349.Google Scholar
  21. 21.
    Alper, C. A., Colten, H. R., Gear, J. S. S., Rabson, A. R., and Rosen, F. S., 1976, Homozygous human C3 deficiency: the role of C3 in antibody production, CT-induced vasopermeability and cobra venom-induced passive hemolysis, J. Clin. Invest. 57: 222.PubMedCentralPubMedGoogle Scholar
  22. 22.
    Alper, C. A., Colten, H. R., Rosen, F. S., Rabson, A. R., Macnab, G. M., and Gear, J. S. S., 1972, Homozygous deficiency of C3 in a patient with repeated infections, Lancet 11:1179.Google Scholar
  23. 23.
    Alper, C. A., Azen, E. A., Geserick, G., Goedde, H. W., Rittner, Ch., Teisberg, P., and Wieme, R., 1973, Statement on the polymorphism of the third component of complement in man (C3), Vox Sang. 25: 18.Google Scholar
  24. 24.
    Arroyave, C. M., Personal communication.Google Scholar
  25. 25.
    Arroyave, C. M., Levy, R. M., and Johnson, J. S., 1975, Genetic deficiency of the fourth component of complement in Wistar rats (Abstract), Federation Proc. 33: 795.Google Scholar
  26. 26.
    Arvilommi, H. A., 1972, Studies on serum Pi-and C3-polymorphism in Finland, Acta Pathol. Microhiol. Scand. Suppl.:234.Google Scholar
  27. 27.
    Arvilommi, H., 1974, Capacity of complement C3 phenotypes to bind on to mononuclear cells in man, Nature 251: 740.PubMedGoogle Scholar
  28. 28.
    Arvilommi, H., Berg, K., and Eriksson, A. W., 1973, C3 types and their inheritance in Finnish Lapps, Maris (Cheremisses) and Greenland Eskimos, Humangenetik 18: 253.PubMedGoogle Scholar
  29. 29.
    Austen, K. F., and Sheffer, A. L., 1965, Detection of hereditary angioneurotic edema by demonstration of a reduction in the second component of human complement, N. Engl. J. Med. 272: 649.PubMedGoogle Scholar
  30. 30.
    Azen, E. A., and Cooper, N. R., 1971, Characterization of C4 in acid—urea starch gel system (Abstract), Clin. Res. 19: 437.Google Scholar
  31. 31.
    Azen, E. A., and Smithies, O., 1968, Genetic polymorphism of C’3 (ß,c-globulin) in human serum, Science 162: 905.PubMedGoogle Scholar
  32. 32.
    Azen, E. A., Smithies, O., and Hiller, O., 1969, High-voltage starch-gel electrophoresis in the study of post-albumin proteins and C’3 (ß„-globulin) polymorphism, Biochem. Genet. 3: 215.PubMedGoogle Scholar
  33. 33.
    Bach, S., Ruddy, S., and Austen, K. F., 1971, Electrophoretic polymorphism of the fourth component of human complement (C4) in paired maternal and foetal plasmas, Immunology 21: 869.PubMedCentralPubMedGoogle Scholar
  34. 34.
    Ballow, M., Shira, J. E., Harden, L., Yang, S. Y., and Day, N. K., 1975, Complete absence of the third component of complement in man, J. Clin. Invest. 56: 703.PubMedCentralPubMedGoogle Scholar
  35. 35.
    Beckman, L., Brönnestam, R., Cedergren, B., and Uden, S., 1974, HL-A antigens, blood groups, serum groups, and red cell enzyme types in psoriasis, Hum. Hered. 24: 496.PubMedGoogle Scholar
  36. 36.
    Berg, K., and Eriksson, A. W., 1973, Genetic marker systems in Arctic populations. VI. Polymorphism of C3 in Icelanders, Hum. Hered. 23: 247.PubMedGoogle Scholar
  37. 37.
    Berg, K., Arvilommi, H., and Eriksson, A. W., 1972, Genetic marker systems in Arctic populations, Hum. Hered. 22: 481.Google Scholar
  38. 38.
    Berg, K., Noer, G., Heiberg, A., 1970, Studies on the genetic polymorphism of the C’3 of human serum, Clin. Genet. 1: 177.Google Scholar
  39. 39.
    Biro, C. E., 1966, The role of C6 in some types of hypersensitivity, Immunology 10: 563.PubMedCentralPubMedGoogle Scholar
  40. 40.
    Biro, C. E., and Ortega, M. L., 1966, Algunas características del sexto componente del complemento, Arch. Inst. Cardiol. Mex. 36: 166.PubMedGoogle Scholar
  41. 41.
    Boyer, J. T., Gall, E. P., Norman, M. E., Nilsson, U. R., and Zimmerman, T. S., 1975, Hereditary deficiency of the seventh component of complement, J. Clin. Invest. 56: 905.PubMedCentralPubMedGoogle Scholar
  42. 42.
    Brönnestam, R., 1973, Studies of the C3 polymorphism. Relationship between phenotype and quantitative immunochemical measurements, Hum. Hered. 23: 128.PubMedGoogle Scholar
  43. 43.
    Brönnestam, R., 1973, Studies of the C3 polymorphism. Relationship between C3 phenotypes and rheumatoid arthritis, Hum. Hered. 23: 206.PubMedGoogle Scholar
  44. 44.
    Brönnestam, R., 1973, Studies of the C3 polymorphism. Distribution of C3 phenotypes in different areas of Sweden, Hum. Hered. 23: 361.PubMedGoogle Scholar
  45. 45.
    Brönnestam, R., and Cedergren, B., 1972, Studies of the C3 polymorphism. Relationship between C3 phenotypes and antibody titres, Hum. Hered. 23: 214.Google Scholar
  46. 46.
    Brönnestam, R., Beckman, L., and Cedergren, B., 1971, Genetic polymorphism of the complement component C3 in Swedish Lapps, Hum. Hered. 21: 267.PubMedGoogle Scholar
  47. 47.
    Caren, L. D., and Rosenberg, L. T., 1965, Complement in skin grafting in mice, Immunology 9: 359.PubMedCentralPubMedGoogle Scholar
  48. 48.
    Caren, L. D., and Rosenberg, L. T., 1966, The role of complement in resistance to endogenous and exogenous infection with a common mouse pathogen, Corynebacterium kutscheri, J. Exp. Med. 124: 689.PubMedCentralPubMedGoogle Scholar
  49. 49.
    Carpenter, C. B., Ruddy, S., Shehadeh, I. H., Müller-Eberhard, H. J., Merrill, J. P., and Austen, K. F., 1969, Complement metabolism in man: hypermetabolism of the fourth (C4) and third (C3) components in patients with renal allograft rejection and hereditary angioedema, J. Clin. Invest. 48: 1495.PubMedCentralPubMedGoogle Scholar
  50. 50.
    Champion, R. H., and Lachmann, P. J., 1969, Hereditary angioedema treated with eaminocaproic acid, Br. J. Dermatol. 81: 763.PubMedGoogle Scholar
  51. 51.
    Cinader, B., and Dubiski, S., 1963, An alpha-globulin allotype in the mouse (MuBI), Nature 200: 781.PubMedGoogle Scholar
  52. 52.
    Cinader, B., Dubiski, S., and Wardlaw, A. C., 1964, Distribution, inheritance, and properties of an antigen, MuBI, and its relation to hemolytic complement, J. Exp. Med. 120: 897.PubMedCentralPubMedGoogle Scholar
  53. 53.
    Cinader, B., Jeejeebhoy, H. F., Koh, S. W., and Rabbat, A. G., 1971, Immunosuppressive and graft-rejecting antibodies in heterologous anti-lymphocytic serum, J. Exp. Med. 133: 81.PubMedCentralPubMedGoogle Scholar
  54. 54.
    Colten, H. R., 1972, Ontogeny of the human complement system: in vitro biosynthesis of individual complement components by fetal tissue, J. Clin. Invest. 51: 725.PubMedCentralPubMedGoogle Scholar
  55. 55.
    Colten, H. R., 1972, In vitro synthesis of a regulator of mammalian gene expression, Proc. Natl. Acad. Sci. U.S.A. 69: 2233.PubMedCentralPubMedGoogle Scholar
  56. 56.
    Colten, H. R., and Alper. C. A., 1972, Hemolytic efficiencies of genetic variants of human C3, J. Immunol. 108: 1184.PubMedGoogle Scholar
  57. 57.
    Colten, H. R., and Frank, M. M., 1972, Biosynthesis of the second and fourth components of complement in vitro by tissues isolated from guinea pigs with genetically determined C4 deficiency, Immunology 22: 991.PubMedCentralPubMedGoogle Scholar
  58. 58.
    Cotten, H. R., and Parkman, R., 1972. Biosynthesis of the fourth component of complement (C4) by C4-deficient guinea pig—HeLa cell hybrids, Science 176: 1029.Google Scholar
  59. 59.
    Colten, H. R., Borsos, T., and Rapp, H. J., 1970, Isoelectric focusing of human and guinea pig C2: polymorphism of guinea pig C2, Immunology 18: 467.PubMedCentralPubMedGoogle Scholar
  60. 60.
    Cooper, N. R., ten Bensel, R., and Kohler, P. F., 1968, Studies of an additional kindred with hereditary deficiency of the second component of human complement (C2) and description of a new method for the quantitation of C2, J. Immunol. 101: 1176.PubMedGoogle Scholar
  61. 61.
    Crisler, C., and Frank, M. M., 1965, Skin graft rejection and the Arthus reaction in mice deficient in the third component (C’3) of complement (Abstract), Federation Proc. 24: 447.Google Scholar
  62. 62.
    Davis, J. S., IV, Personal communication.Google Scholar
  63. 63.
    Day, N. K., Geiger, H., McLean, R., Michael, A., and Good. R. A., 1973, C2 deficiency: development of lupus erythematosus, J. Clin. Invest. 52: 1601.PubMedCentralPubMedGoogle Scholar
  64. 64.
    Day, N. K., Geiger, H., Stroud, R., deBracco, M., Mancado, B., Windhorst, D., and Good, R. A., 1972, C 1 deficiency: an inborn error associated with cutaneous and renal disease, J. Clin. Invest. 51: 1102.PubMedCentralPubMedGoogle Scholar
  65. 65.
    Day, N. K., L’Esperance, P., Good, R. A., Michael, A. F., Hansen. J. A., Dupont, B., and Jersild, C., 1975, Hereditary C2 deficiency: genetic studies and association with the HL-A system, J. Exp. Med. 141: 1464.Google Scholar
  66. 66.
    DeBracco, M. M. E., Windhorst, D., Stroud, R. M., and Moncada, B., 1974, The autosomal recessive mode of inheritance of C1r deficiency in a large Puerto Rican family, Clin. Exp. Immunol. 16: 183.Google Scholar
  67. 67.
    Démant, P., Capkokd, J., Hinzovd, E., and Vorâcovd, B., 1973. The role of the histocompatibility-2-linked Ss—Slp region in the control of mouse complement, Proc. Natl. Acad. Sci. U.S.A. 70: 863.Google Scholar
  68. 68.
    Dissing, J., and Sorensen, H., 1971, Studies on C’3 polymorphism in Denmark, Hum. Hered. 21: 272.PubMedGoogle Scholar
  69. 69.
    Dissing, J., Lund, J., and Sorensen, H., 1972, C3 polymorphism in a group of old arteriosclerotic patients, Hum. Hered. 22: 466.PubMedGoogle Scholar
  70. 70.
    Donaldson, V. H., 1968, Mechanisms of activation of C’1 esterase in hereditary angioneurotic edema plasma in vitro. The role of Hageman factor, a clot-promoting agent, J. Exp. Med. 127: 411.PubMedCentralPubMedGoogle Scholar
  71. 71.
    Donaldson, V. H., and Evans, R. R., 1963, A biochemical abnormality in hereditary angioneurotic edema: absence of a serum inhibitor of C1-esterase, Am. J. Med. 35: 37.PubMedGoogle Scholar
  72. 72.
    Donaldson, V. H., and Rosen, F. S., 1964, Action of complement in hereditary angioneurotic edema: the role of C’ 1 esterase, J. Clin. Invest. 43: 2204.PubMedCentralPubMedGoogle Scholar
  73. 73.
    Donaldson, V. H., and Rosen, F. S., 1966, Hereditary angioneurotic edema. A clinical survey, Pediatrics 37: 1017.PubMedGoogle Scholar
  74. 74.
    Donaldson, V., and Stroud, R. M., 1974, Genetic defects of complement, in “Progress in Immunology II,” Vol. 1 (L. Brent and J. Holborow, eds.), p. 290, North-Holland Publishing Company, Amsterdam.Google Scholar
  75. 75.
    Donaldson, V. H., Ratnoff, O. D., Dias da Silva, W., and Rosen, F. S., 1969, Permeability-increasing activity in hereditary angioneurotic edema: mechanism of formation and partial characterization, J. Clin. Invest. 48: 642.PubMedCentralPubMedGoogle Scholar
  76. 76.
    Dubiski, S., and Cinader, B., 1966, Gene dosage effect of serum concentration of a complement component, MuBI, Proc. Soc. Exp. Biol. Med. 122: 775.PubMedGoogle Scholar
  77. 77.
    Ecker, E. E., 1921, Quantitative relations between amboceptor and the serum of complement deficient guinea pigs, J. Infect. Dis. 29: 611.Google Scholar
  78. 78.
    Einstein, L. P., Alper, C. A., Bloch, K. J., Herrin, J. T., Rosen, F. S., David, J. R., and Colten, H. R., 1975, Biosynthetic defect in monocytes from human beings with genetic deficiency of the second component of complement, N. Engl. J. Med. 292: 1169.Google Scholar
  79. 79.
    Ellman, L., Green, I., and Frank, M., 1970, Genetically controlled total deficiency of the fourth component of complement in the guinea pig, Science 170: 74.PubMedGoogle Scholar
  80. 80.
    Ellman, L., Green, I., Judge, F., and Frank, M. M., 1972, In vivo studies in C4-deficient guinea pigs, J. Exp. Med. 134: 162.Google Scholar
  81. 81.
    Erickson, R. P., Tachibana, D. K., Herzenberg, L. A., and Rosenberg, L. T., 1964, A single gene controlling hemolytic complement and a serum antigen in the mouse, J. Immunol. 92: 611.PubMedGoogle Scholar
  82. 82.
    Farhud, D. D., and Walter, H., 1973, Verwendung des C3 (/31 -globulin)—Polymorphismus bei der Vaterschaftsbegutachtung, Z. Rechtsmed. 72: 225.PubMedGoogle Scholar
  83. 83.
    Farhud, D. D., and Walter, H., 1973, Polymorphism of C’3 in German, Bulgarian, Iranian and Angola population, Humangenetik 17:161.PubMedGoogle Scholar
  84. 84.
    Farhud, D. D., Ananthakrishnan, R., and Walter, H., 1972, Association between C’3 phenotypes and various diseases, Humangenetik 17: 57.PubMedGoogle Scholar
  85. 85.
    Ferreira, A., and Nussenzweig, V., 1975, Genetic control of complement (C3) levels and of the development of the lymphoid system (Abstract), Federation Proc. 34: 979.Google Scholar
  86. 86.
    Forbes, C. D., Pensky, J., and Ratnoff, O. D., 1970, Inhibition of activated Hageman factor and activated thromboplastin antecedent by purified serum CI inactivator, J. Lab. Clin. Med. 76: 809.PubMedGoogle Scholar
  87. 87.
    Frank, M. M., May, J. E., and Kane, M. A., 1973, Contributions of the classical and alternate pathways to the biological effects of endotoxin, J. Infect. Dis. 128: 5176.Google Scholar
  88. 88.
    Frank, M. M., May, J., Gaither, T., and Ellman, L., 1971, In vitro studies of complement function in sera of C4-deficient guinea pigs, J. Exp. Med. 134: 176.Google Scholar
  89. 89.
    Frank, M. M., Sergent, J. S., Kane, M. A., and Ailing, D. W., 1972, Epsilon aminocaproic acid therapy of hereditary angioneurotic edema. A double-blind study, N. Engl. J. Med. 286: 808.PubMedGoogle Scholar
  90. 90.
    Fraser, G. R., Volkers, W. S., Bernini, L. F., De Greve, W. B., van Loghem, E., Meera Khan, P., Nijenhuis, L. E., Veltkamp, J. J., Vogel, G. P., and Went, L. N., 1974, A search for associations between genetical polymorphic systems and physical, biochemical and hematological variables, Hum. Hered. 24: 424.PubMedGoogle Scholar
  91. 91.
    Fu, S. M., Kunkel, H. G., Brusman, H. P., Allen, F. H., Jr., and Fortino, M., 1974, Evidence for linkage between HL-A histocompatibility genes and those involved in synthesis of the second component of complement, J. Exp. Med. 140: 1108.PubMedCentralPubMedGoogle Scholar
  92. 92.
    Gedde-Dahl, T., Jr., Teisberg, P., and Thorsby, E., 1974, C3 polymorphism: genetic linkage relations, Clin. Genet. 6: 66.PubMedGoogle Scholar
  93. 93.
    Gelfand, E. W., Clarkson, J. D., and Minta, J. O., 1975, Selective deficiency of the second component of complement in a patient with anaphylactoid purpura, Clin. Immunol. Immunopathol. 4: 269.PubMedGoogle Scholar
  94. 94.
    Geserick, G., Rose, M., and Schnitzler, St., 1970, Untersuchungen zur Identität des polymorphen Serumproteins Pt, Z. Immunitaetsforsch. Allerg. klin. Immunol. 140: 54.Google Scholar
  95. 95.
    Geserick, G., Marth, H., Schnitzler, St., Gögochia, S. D., Mirvis, A. B., and Annenkow, H. A., 1971, Pt-Typenverteilung bei einem Bevölkerungsisolat im abchasischen Swanetien (Grusinien), Acta Biol. Med. Ger. 26: 411.PubMedGoogle Scholar
  96. 96.
    Gigli, I., Ruddy, S., and Austen, K. F., 1968, The stoichiometric measurement of the serum inhibitor of the first component of complement by the inhibition of immune hemolysis, J. Immunol. 100: 1154.PubMedGoogle Scholar
  97. 97.
    Gigli, I., Mason, J. W., Colman, R. W., and Austen, K. F., 1970, Interaction of plasma kallikrein with the Cl inhibitor, J. Immunol. 104: 574.PubMedGoogle Scholar
  98. 98.
    Glahs, G., 1974, Der Polymorphismus der Komplement-Komponente C’3 in Österreich, Med. Lab. (Stuttg.) 27: 53.Google Scholar
  99. 99.
    Glass, D., Gibson, D. J., Carpenter, C. B., and Schur, P. H., 1976, Hereditary C2 deficiency: HLA gene complex associations with recombinant events (Abstract), J. Immunol. 116: 1734.Google Scholar
  100. 100.
    Glynn, A. A., and Medhurst, F. A., 1967, Possible extracellular and intracellular bactericidal actions of mouse complement, Nature 213: 608.PubMedGoogle Scholar
  101. 101.
    Goedde, H. W., Benkmann, H.-G., Agarwal, D. P., and Hirth, L., 1972, Further investigations on inherited polymorphism of the third component of complement (ß, C/ß, A-globulin), Hum. Hered. 22: 488.PubMedGoogle Scholar
  102. 102.
    Goedde, H. W., Hirth, L., Benkmann, H.-G., Pellicer, A., Pellicer, T., Stahn, M., Suigh, S., 1973, Population genetic studies and serum protein polymorphisms in four Spanish populations, Hum. Hered. 23: 135.PubMedGoogle Scholar
  103. 103.
    Goldman, M. B., and Goldman, J. N., 1975, Relationship of levels of early components of complement to the H-2 complex of mice (Abstract), Federation Proc. 34: 979.Google Scholar
  104. 104.
    Granerus, G., Hallberg, L., Laurel], A. B., and Wetterquist, H., 1967, Studies on histamine metabolism and the complement system in hereditary angioneurotic edema, Acta Med. Scand. 182: 11.PubMedGoogle Scholar
  105. 105.
    Grace, H. J., and Vos, G. H., Personal communication.Google Scholar
  106. 106.
    Greitz, H., Reutter, W., and Schmutzler, W., 1964, Release of histamine from rabbit blood cells by an anaphylatoxin-like substance formed in normal and C’3 defect rabbit plasma, Nature 202: 1346.Google Scholar
  107. 107.
    Hansen, T. H., Krasteff, T. N., and Shreffler, D. C., 1974, Quantitative variations in the expression of the mouse serum antigen Ss and its sex-limited allotype SIp, Biochem. Genet. 12: 281.PubMedGoogle Scholar
  108. 108.
    Hansen, T. H., Shin, H. S., and Shreffler, D. C., 1975, Evidence for the involvement of the Ss protein of the mouse in the hemolytic complement system, J. Exp Med. 141: 1216.PubMedGoogle Scholar
  109. 109.
    Harpel, P. C., and Cooper, N. R., 1975, Studies on human plasma CT inactivatorenzyme interactions. I. Mechanisms of interaction with Cls, plasmin, and trypsin, J. Clin. Invest. 55: 593.PubMedCentralPubMedGoogle Scholar
  110. 110.
    Harpel, P. C., Hugli, T. E., and Cooper, N. R., 1975, Studies on human plasma CT inactivator—enzyme interactions. II. Structural features of an abnormal Cl inactivator from a kindred with hereditary angioneurotic edema, J. Clin. Invest. 55: 605.PubMedCentralPubMedGoogle Scholar
  111. 111.
    Hauptmann, G., Grosshans, E., Heid, E., Mayer, S., and Basset, A., 1974, Lupus érythémateux aigu avec déficit complet de la fraction C4 du complément, Nouv. Presse Med. 3: 881.PubMedGoogle Scholar
  112. 112.
    Heusinkveld, R. S., Leddy, J. P., Klemperer, M. R., and Breckenridge, R. T., 1974, Hereditary deficiency of the sixth component of complement in man, J. Clin. Invest. 53: 554.PubMedCentralPubMedGoogle Scholar
  113. 113.
    Hinzovd, E., Démant, P., and Ivânyi, P., 1972, Genetic control of haemolytic complement in mice: association with H-2, Folio Biol. (Prague) 18: 237.Google Scholar
  114. 114.
    Hobart, M. J., Lachmann, P. J., and Alper, C. A., 1975, Polymorphism of human C6, in “Protides of the Biological Fluids,” Vol. 22 (H. Peeters, ed.), pp. 575–580, Pergamon Press, New York.Google Scholar
  115. 115.
    Howie, J. B., and Helyer, B. J., 1965, Autoimmune disease in mice, Ann. N.Y. Acad. Sci. 124: 167.PubMedGoogle Scholar
  116. 116.
    Hrdy, D. B., Barnicot, N. A., and Alper, C. A., 1975, Protein polymorphism in the Hanuman langur (Presbytis entellus), Folio Primatol. 24: 173.Google Scholar
  117. 117.
    Hyde, R. R., 1923, Complement-deficient guinea-pig serum, J. Immunol. 8: 267.Google Scholar
  118. 118.
    Hyde, R. R., 1932, The complement deficient guinea pig: a study of an inheritable factor in immunity, Am. J. Hvg. 15: 824.Google Scholar
  119. 119.
    Johnson, A. M., Alper, C. A., Rosen, F. S., and Craig, J. M., 1971, CT inhibitor: evidence for decreased hepatic synthesis in hereditary angioneurotic edema, Science 173: 553.PubMedGoogle Scholar
  120. 120.
    Johnson, K. J., and Ward, P. A., 1971, Protective function of C6 in rabbits treated with bacterial endotoxin, J. Immunol. 106: 1125.PubMedGoogle Scholar
  121. 121.
    Johnston, R. B., Jr., Klemperer, M. R., Alper, C. A., and Rosen, F. S., 1969, The enhancement of bacterial phagocytosis by serum. The role of complement components and two cofactors, J. Exp. Med. 129: 1275.PubMedCentralPubMedGoogle Scholar
  122. 122.
    Kane, M. A., May, J. E., and Frank, M. M., 1973, Interactions of the classical and alternate complement pathway with endotoxin lipopolysaccharide. Effect on platelets and blood coagulation, J. Clin. Invest. 52: 370.PubMedCentralPubMedGoogle Scholar
  123. 123.
    Klemperer, M. R., 1969, Hereditary deficiency of the second component of complement in man: an immunochemical study, J. Immunol. 102: 168.PubMedGoogle Scholar
  124. 124.
    Klemperer, M. R., Austen, K. F., and Rosen, F. S., 1967, Hereditary deficiency of the second component of complement (C’2) in man, J. Immunol. 98: 72.PubMedGoogle Scholar
  125. 125.
    Klemperer, M. R., Donaldson, V. H., and Rosen, F. S., 1968, Effect of C’ 1 esterase on vascular permeability in man: studies in normal and complement-deficient individuals and in patients with hereditary angioneurotic edema, J. Clin. Invest. 47: 604.PubMedCentralPubMedGoogle Scholar
  126. 126.
    Klemperer, M. R., Woodworth, H. C., Rosen, F. S., and Austen, K. F., 1966, Hereditary deficiency of the second component of complement in man, J. Cin. Invest. 45: 880.Google Scholar
  127. 127.
    Kohler, P. F., Percy, J., Campion, W. M., and Smyth, C. J., 1974, Hereditary angioedema and “familial” lupus erythematosus in identical twin boys, Am. J. Med. 56: 406.PubMedGoogle Scholar
  128. 128.
    Kühnl, P., and Spielmann, W., 1972, Untersuchungen zum C’3-Polymorphismus (Ow-globulin), Humangenetik 15: 7.PubMedGoogle Scholar
  129. 129.
    Lachmann, P. J., 1970, C6-deficiency in rabbits, in “Protides of the Biological Fluids,” Vol. 17 (H. Peeters, ed.), pp. 301–309, Pergamon Press, Oxford.Google Scholar
  130. 130.
    Landerman, N. S., 1962, Hereditary angioneurotic edema. I. A case report and review of the literature, J. Allergy 33: 316.PubMedGoogle Scholar
  131. 131.
    Landerman, N. S., Webster, M. E., Becker, E. L., and Ratcliffe, H. E., 1962, Hereditary angioneurotic edema. II. Deficiency of inhibitor for serum globulin permeability factor and/or plasma kallikrein, J. Allergy 33: 330.PubMedGoogle Scholar
  132. 132.
    Laurell, A.-B., and Martensson, U., 1971, CT inactivator protein complexed with albumin in plasma from a patient with angioneurotic edema, Eur. J. Immunol. 1: 146.PubMedGoogle Scholar
  133. 133.
    Laurell, A. B., Lindegren, J., Malmros, I., and Martensson. H., 1969, Enzymatic and immunochemical estimation of C 1 esterase inhibitor in sera from patients of hereditary angioneurotic edema, Scand. J. Clin. Invest. 24: 221.PubMedGoogle Scholar
  134. 134.
    Leddy, J. P., Griggs, R. C., Klemperer, M. R., and Frank, M. M., 1975, Hereditary complement (C2) deficiency with dermatomyositis, Am. J. Med. 58: 83.PubMedGoogle Scholar
  135. 135.
    Leddy, J. P., Frank, M. M., Gaither, T., Baum, J., and Klemperer, M. R., 1974, Hereditary deficiency of the sixth component of complement in man. I. Immunochemical, biologic, and family studies, J. Clin. Invest. 53: 544.PubMedCentralPubMedGoogle Scholar
  136. 136.
    Levy, N. L., and Ladda, R. L., 1971, Restoration of hemolytic complement activity in CS-deficient mice by gene complementation in hybrid cells, Nature (London) New Biol. 229: 51.Google Scholar
  137. 137.
    Levy, N. L., Synderman, R., Ladda, R. L., and 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. U.S.A. 70: 3125.PubMedCentralPubMedGoogle Scholar
  138. 138.
    Lindberg, L. H., and Rosenberg, L. T., 1968, Nephrotoxic serum nephritis in mice with a genetic deficiency in complement, J. Immunol. 100: 34.PubMedGoogle Scholar
  139. 139.
    Lundh, B., Laurell, A. B., Wetterqvist, H., White, T., and Granerus, G., 1968, Case report: a case of hereditary angioneurotic oedema successfully treated with epsilonaminocaproic acid. Studies on C’ 1 esterase inhibitor, C’ 1 activation, plasminogen level and histamine metabolism, Clin. Exp. Immunol. 3: 733.PubMedCentralPubMedGoogle Scholar
  140. 140.
    Mauff, G., Potrafki, B. G., Freis, H., and Pulverer, G., 1974, Vergleichende Untersuchungen zum Polymorphismus des Posttransferrins (Pt) and der dritten Komponente des Human Komplements (C3), Humangenetik 21: 75.PubMedGoogle Scholar
  141. 141.
    May, J. E., and Frank, M. M., 1972, Complement-mediated tissue damage: contribution of the classical and alternate complement pathways in the Forssman reaction, J. Immunol. 108: 1517.PubMedGoogle Scholar
  142. 142.
    May, J. E., Green, I., and Frank, M. M., 1972, The alternate complement pathway in cell damage: antibody-mediated cytolysis of erythrocytes and nucleated cells, J. Immunol. 109: 595.PubMedGoogle Scholar
  143. 143.
    May, J. E., Kane, M. A., and Frank, M. M., 1972, Host defense against bacterial endotoxemia: contribution of the early and late components of complement to detoxification, J. Immunol. 109: 893.PubMedGoogle Scholar
  144. 144.
    May, J. E., Kane, M. A., and Frank, M. M., 1972, Immune adherence by the alternate complement pathway, Proc. Soc. Exp. Biol. Med. 141: 287.PubMedGoogle Scholar
  145. 145.
    Meo, T., Krasteff, T., and Shreffler, D. C., 1975, Immunochemical characterization of murine H-2 controlled Ss (serum substance) protein through the identification of its human homologue as the fourth component of complement, Proc. Natl. Acad. Sci. U.S.A. 72: 4536.PubMedCentralPubMedGoogle Scholar
  146. 146.
    Miller, G. W., and Nussenzweig, V., 1975, A new complement function: solubilization of antigen—antibody aggregates, Proc. Natl. Acad. Sci. U.S.A. 72: 418.PubMedCentralPubMedGoogle Scholar
  147. 147.
    Moncada, B., Day, N. K., Good, R. A., and Windhorst, D. B., 1972, Lupuserythematosus-like syndrome with a familial defect of complement, N. Engl. J. Med. 286: 689.PubMedGoogle Scholar
  148. 148.
    Moore, H. D., 1919, Complementary and opsonic functions in their relation to immunity. A study of the serum of guinea-pigs naturally deficient in complement, J. Immunol. 4: 425.Google Scholar
  149. 149.
    Müller-Eberhard, H. J., 1975, Complement, in “Annual Review of Biochemistry,” Vol. 44 (E. E. Snell, P. D. Boyer, A. Meister, and C. C. Richardson, eds.), pp. 697–724, Annual Reviews Inc., California.Google Scholar
  150. 150.
    Nelson, R. A., and Biro, C. E., 1968, Complement components of a hemolytically deficient strain of rabbits, Immunology 14: 527.PubMedCentralPubMedGoogle Scholar
  151. 151.
    Ochs, H. D., Jackson, C. G., and Wedgwood, R. J., Personal communication.Google Scholar
  152. 152.
    Okada, N., and Rosenberg, L. T., 1964, T2 phage neutralizing activity of mouse serum (Abstract), Bacteriol. Proc. 64: 141.Google Scholar
  153. 153.
    Oster, W., 1888, Hereditary angioneurotic edema, Arn. J. Doled. Sci. 95: 362.Google Scholar
  154. 154.
    Osterlund, C. K., Espinoza, L., Parker, L. P., and Schur, P. H., 1975, Inherited C2 deficiency and systemic lupus erythematosus: studies on a family, Ann. Intern. Med. 82: 323.Google Scholar
  155. 155.
    Passmore, H. C., and Shreffler, D. C., 1970, A sex-limited serum protein variant in the mouse: inheritance and association with the H-2 region, Biochem. Genet. 4: 351.PubMedGoogle Scholar
  156. 156.
    Pensky, J., and Schwick, H. G., 1969, Human serum inhibitor of Cl esterase: identity with alpha 2-neuraminoglycoprotein, Science 163: 698.PubMedGoogle Scholar
  157. 157.
    Pensky, J., Levy, L. R., and Lepow, I. H., 1961, Partial purification of a serum inhibitor of C’ 1 esterase, J. Biol. Chem. 236: 1674.PubMedGoogle Scholar
  158. 158.
    Peters, D. K., 1974, Alternative pathways of complement activation, in “Progress in Immunology II,” Vol. I (L. Brent and J. Holborow, eds.), p. 297, North-Holland Publishing Company, Amsterdam.Google Scholar
  159. 159.
    Petersen, B. H., Graham, J. A., and Boroks, G. F., 1975, Human deficiency of the 8th component of complement (Abstract), Clin. Res. 23: 294A.Google Scholar
  160. 160.
    Phillips, M. E., Rother, U. A., Rother, K. O., and Thorbecke, G. J., 1969, Studies on the serum proteins of chimeras. III. Detection of donor type C’5 in allogeneic and congenic post-irradiation chimeras, Immunology 17: 315.PubMedCentralPubMedGoogle Scholar
  161. 161.
    Pickering, R. J., Kelly, J. R., Good, R. A., and Gewurz, H., 1969, Replacement therapy in hereditary angioedema. Successful treatment of two patients with fresh frozen plasma, Lancet 1: 326.PubMedGoogle Scholar
  162. 162.
    Pillemer, L., Blum, L., Lepow, I. H., Ross, O. A., Todd. E. W., and Wardlaw, A. C., 1954, The properdin system and immunity. I. Demonstration and isolation of a new serum protein, properdin, and its role in immune pheomena, Science 120: 279.Google Scholar
  163. 163.
    Polley, M. J., 1968, Inherited C’2 deficiency in man: lack of immunochemically detectable C’2 protein in serums from deficient individuals, Science 161: 1149.PubMedGoogle Scholar
  164. 164.
    Prokop, O., Rose, H., and Geserick, G., 1970, Die Anwendung des Posttransferrin Polymorphismus (Pt) in der Vaterschaftsbegutachtung, Dtsch. Gesundheitwes. 25: 1910.Google Scholar
  165. 165.
    Propp, R. P., and Alper, C. A., 1968, C’3 synthesis in the human fetus and lack of transplacental passage, Science 162: 672.PubMedGoogle Scholar
  166. 166.
    Quincke, H., 1882, Über akutes umschriebenes Hautödem, Monatsh. Prak. Dermat. 1: 129.Google Scholar
  167. 167.
    Ratnoff, O. D., Pensky, J., Ogston, D., and Naff, G. B., 1969, The inhibition of plasmin, plasma kallikrein, plasma permeability factor, and the C’ Ir subcomponent of the first component of complement by serum C’ 1 esterase inhibitor, J. Exp. Med. 129: 315.PubMedCentralPubMedGoogle Scholar
  168. 168.
    Rittner, Ch., and Hauptmann, G., Personal communication.Google Scholar
  169. 169.
    Rittner, Ch., and Rittner, B., 1973, Description of three hitherto unknown C3 variants: F 0.6, F 0.7 and S 2.4 (old nomenclature), included in the workshop (Abstract), Vox Sang. 25: 17.Google Scholar
  170. 170.
    Rittner, Ch., Grosse-Wilde, H., Rittner, B., Netzel, B., Scholz, S., Lorenz, H., and Albert, E. D., 1975, Linkage group HL-A-MLC-BF (Properdin Factor B). The site of the Bf locus at the immunogenetic linkage group on chromosome 6, Humangenetik 27: 173.PubMedGoogle Scholar
  171. 171.
    Root, R. K., Ellman, L., and Frank, M. M., 1972, Bactericidal and opsonic properties of C4-deficient guinea pig serum, J. Immunol. 109: 477.PubMedGoogle Scholar
  172. 172.
    Rose, M., and Geserick, G., 1969, Ein neuer Serumproteinpolymorphismus: Pt. Erste Hinweise für eine genetische Steuerung, Acta Biol. Med. Ger. 23: 351.PubMedGoogle Scholar
  173. 173.
    Rosen, F. S., Charache, P., Donaldson, V., and Pensky, J., 1965, Hereditary angioneurotic edema: two genetic variants, Science 148: 957.PubMedGoogle Scholar
  174. 174.
    Rosen, F. S., Alper, C. A., Pensky, J., Klemperer, M. R.. and Donaldson, V. H., 1971, Genetically determined heterogeneity of the CI esterase inhibitor in patients with hereditary angioneurotic edema, J. Clin. Invest. 50: 2143.PubMedCentralPubMedGoogle Scholar
  175. 175.
    Rosenfeld, S. I., and Leddy, J. P., 1974, Hereditary deficiency of fifth component of complement (C5) in man (Abstract), J. Clin. Invest. 53: 67a.Google Scholar
  176. 176.
    Rosenfeld, S. I., Ruddy, S., and Austen, K. F., 1969, Structural polymorphism of the fourth component of human complement, J. Clin. Invest. 48: 2283.PubMedCentralPubMedGoogle Scholar
  177. 177.
    Rother, K., and Rother, U., 1965, Studies on complement defective rabbits. IV. Blood clearance of intravenously injected S. typhi by the reticuloendothelial system, Proc. Soc. Exp. Biol. Med. 119: 1055.PubMedGoogle Scholar
  178. 178.
    Rother, K., Rother, U., and Schindera, F., 1964, Passive Arthus-Reaktion bei Komplement-Defekt Kaninchen, Z. Immunitaetsforsch. exp. Ther. 126: 473.Google Scholar
  179. 179.
    Rother, K., Rother, U., Müller-Eberhard, H. J., and Nilsson, U. R., 1966, Deficiency of C6 in rabbits with an inherited complement defect, J. Exp. Med. 124: 773.PubMedCentralPubMedGoogle Scholar
  180. 180.
    Rother, K., Rother, U., Vassali, P., and McCluskey, R. T., 1967, Nephrotoxic serum nephritis in C’6-deficient rabbits. I. Study of the second phase of the disease, J. Immunol. 98: 965.PubMedGoogle Scholar
  181. 181.
    Rother, U., and Rother, K., 1961, Ober einen eingeborenen Komplement-Defekt bei Kaninchen, Z. Immunitaetsforsch. exp. Ther. 121: 224.Google Scholar
  182. 182.
    Ruddy, S., and Austen, K. F., 1967, A stoichiometric assay for the fourth component of complement in whole human serum using EAC’laó’ and functionally pure human second component, J. Immunol. 99: 1162.PubMedGoogle Scholar
  183. 183.
    Ruddy, S., Klemperer, M. R., Rosen, F. S., Austen, K. F., and Kumate, J., 1970, Hereditary deficiency of the second component of complement (C2) in man, Immunology 18: 943.PubMedCentralPubMedGoogle Scholar
  184. 184.
    Schreiber, A. D., and Frank, M. M., 1972, Role of antibody and complement in the immune clearance and destruction of erythrocytes. I. In vivo effects of IgG and IgM complement-fixing sites, J. Clin. Invest. 51: 575.Google Scholar
  185. 185.
    Schultze, H. E., Heide, K., and Haupt, H., 1962, Über ein bisher unbekanntes saures alpha 2 Glykoprotein, Naturwissenschaften 49: 133.Google Scholar
  186. 186.
    Seth, S., and Berndt, H., 1974, Genetic polymorphism of the human complement component C’3 among North Germans, Humangenetik 21: 59.PubMedGoogle Scholar
  187. 187.
    Sheffer, A. L., Austen, K. F., and Rosen, F. S., 1972, Tranexamic acid therapy in hereditary angioneurotic edema, N. Engl. J. Med. 287: 452.PubMedGoogle Scholar
  188. 188.
    Sheffer, A. L., Craig, J. M., Willms-Kretschmer, K., Austen, K. F., and Rosen, F. S., 1971, Histopathological and ultrastructural observations on tissue from patients with hereditary angioneurotic edema, J. Allergy 47: 292.PubMedGoogle Scholar
  189. 189.
    Shevach, E., Green, I., and Frank, M. M., 1976, Linkage of C4 deficiency to the major histocompatibility locus in the guinea pig (Abstract), J. Immunol. 116: 1750.Google Scholar
  190. 190.
    Shin, H. S., Smith, M. R., and Wood, W. B., Jr., 1969, Heat labile opsonins to pneumococcus. II. Involvement of C3 and C5, J. Exp. Med. 130: 1229.PubMedCentralPubMedGoogle Scholar
  191. 191.
    Shokeir, M. H., 1973, The genetics of hereditary angioedema: a hypothesis, Clin. Genet. 4: 494.PubMedGoogle Scholar
  192. 192.
    Shreffler, D. C., 1965, The Ss system of the mouse—A quantitative serum protein difference genetically controlled by the H-2 region, in “Isoantigens and Cell Interactions” (J. Palm, ed.), pp. 11–19, Wistar Institute Press, Philadelphia.Google Scholar
  193. 193.
    Shreffler, D. C., and Owen, R. D., 1963, A serologically detected variant in mouse serum: inheritance and association with the Histocompatibility-2 locus, Genetics 48: 9.PubMedCentralPubMedGoogle Scholar
  194. 194.
    Spaulding, W. B., 1960, Methyltestosterone therapy for hereditary episodic edema, Arch. Intern. Med. 53: 739.Google Scholar
  195. 195.
    Stiffel, C., Biozzi, G., Monton, D., Bouthillier, Y., and Decreusefond, C., 1964, Studies on phagocytosis of bacteria by the reticulo-endothelial system in a strain of mice lacking hemolytic complement, J. Immunol. 93: 246.PubMedGoogle Scholar
  196. 196.
    Stossel, T. P., Alper, C. A., and Rosen, F. S., 1973, Serum-dependent phagocytosis of paraffin oil emulsified with bacterial lipopolysaccharide, J. Exp. Med. 137: 690.PubMedCentralPubMedGoogle Scholar
  197. 197.
    Stossel, T. P., Alper, C. A., and Rosen, F. S., 1973, Opsonic activity in the newborn: role of properdin, Pediatrics 134: 173.Google Scholar
  198. 198.
    Stratton, F., Personal communication.Google Scholar
  199. 199.
    Sussman, M., Jones, J. S., Almeida, J. D., and Lachmann, P. J., 1973, Deficiency of second component of complement associated with anaphylactoid purpura and presence of mycoplasma in the serum, Clin. Exp. Immunol. 14: 531.PubMedCentralPubMedGoogle Scholar
  200. 200.
    Tachibana, D. K., Ulrich, M., and Rosenberg, L. T., 1963. The inheritance of hemolytic complement activity in CF-1 mice, J. Immunol. 91: 230.PubMedGoogle Scholar
  201. 201.
    Teisberg, P., 1970, New variants in the C3 system, Hum. Hered. 20: 631.PubMedGoogle Scholar
  202. 202.
    Teisberg, P., 1971, The distribution of C3 types in Norway, Hum. Hered. 21: 154.PubMedGoogle Scholar
  203. 203.
    Teisberg, P., 1971, C3 types of Norwegian Lapps, Hum. Hered. 21: 162.PubMedGoogle Scholar
  204. 204.
    Teisberg, P., 1971, Genetics of the C3 system: family, mother/child, and association studies, Hum. Hered. 21: 458.PubMedGoogle Scholar
  205. 205.
    Teisberg, P., Olaisen, B., Gedde-Dahl, T., Jr., and Thorsby, E., 1975, On the localization of the Gb locus within the MHS region of chromosome no. 6, Tissue Antigens 5: 257.PubMedGoogle Scholar
  206. 206.
    Tobelem, G., Wautier, J. L., and Peltier, A. P., 1974, C2 deficiency, platelet aggregation reduction, and Lobstein’s disease in the same family, Biomed. Express (Paris) 21: 190.Google Scholar
  207. 207.
    Unanue, E. R., Mardiney, M. R., and Dixon, F. J., 1967, Nephrotoxic serum nephritis in complement intact and deficient mice, J. Immnol. 98: 609.Google Scholar
  208. 208.
    Volk, H., Mauersberger, D., Rother, K., and Rother, U., 1964, Prolonged survival of skin homografts in rabbits defective in the third component of complement, Ann. N. Y. Acad. Sci. 120: 26.PubMedGoogle Scholar
  209. 209.
    Ward, P. A., Cochrane, C. G., and Müller-Eberhard, H. J., 1965, The role of serum complement in chemotaxis of leukocytes in vitro, J. Exp. Med. 122: 327.Google Scholar
  210. 210.
    Weitkamp, L. R., Johnston, E., and Guttormsen, S. A., 1974, Probable genetic linkage between loci for Lewis blood-group and complement-C3, Cytogenet. Cell Genet. 13: 183.PubMedGoogle Scholar
  211. 211.
    Wellek, B., and Opferkuch, W., 1975, A case of deficiency of the seventh component of complement in man. Biological properties of a C7-deficient serum and description of a C7-inactivating principle, Clin. Exp. Immunol. 19: 223.PubMedCentralPubMedGoogle Scholar
  212. 212.
    Willms, K., Rosen, F. S., and Donaldson, V. H.. 1975, Observations on the ultrastructure of lesions induced in human and guinea pig skin by esterase and polypeptide from hereditary angioneurotic edema (HANE) plasma, Clin. Immunol. Immunopathol. 4: 174.PubMedGoogle Scholar
  213. 213.
    Wolski, K. P., Schmid, F. R., and Mittal, K. L., 1975, Genetic linkage between HL-A system and a deficit of the second component (C2) of complement, Science 188: 1020.PubMedGoogle Scholar
  214. 214.
    Yang, S. Y., Jensen, R., Folke, L., Good, R. A.. and Day, N. K., 1975, Complement deficiency in hamsters (Abstract), Federation Proc. 33: 795.Google Scholar
  215. 215.
    Ziegler, J. B., Alper, C. A., and Balner, H., 1975, Properdin factor B and histocompatibility loci linked in the rhesus monkey, Nature 254: 609.PubMedGoogle Scholar
  216. 216.
    Ziegler, J. B., Watson, L., and Alper, C. A., 1975, Genetic polymorphism of properdin Factor B in the rhesus: evidence for single subunit structure in primates, J. Immunol. 114: 1649.PubMedGoogle Scholar
  217. 217.
    Ziegler, J. B., Alper, C. A.. Rosen, F. S., Lachmann, P. J., and Sherington, L., 1975, Restoration by purified C3b inactivator of complement-mediated function in vivo in a patient with C3b inactivator deficiency, J. Clin. Invest. 55: 668.PubMedCentralPubMedGoogle Scholar
  218. 218.
    Ziegler, J. B., Rosen, F. S., Alper. C. A., Grupe, W., and Lepow, I. H., 1975. Metabolism of properdin in normal subjects and patients with renal disease, J. Clin. Invest. 56: 761.PubMedCentralPubMedGoogle Scholar
  219. 219.
    Zimmerman, T. S., Arroyave, C. M., and Müller-Eberhard, H. J., 1971, A blood coagulation abnormality in rabbits deficient in the sixth component of complement (C6) and its correction by purified C6, J. Exp. Med. 134: 1591.PubMedCentralPubMedGoogle Scholar
  220. 220.
    Alper, C. A., 1976, Inherited structural polymorphism in human C2. Evidence for genetic linkage between C2 and Bf J. Exp. Med., in press.Google Scholar
  221. 221.
    Blum, L., Lee, K., Lee, S. L., Barone, R., and Wallace, S. L., 1976. Hereditary Cls deficiency (Abstract), Federation Proc. 35: 655.Google Scholar
  222. 222.
    Curman, B., Ostberg, L., Sandberg, L., Malmheden-Eriksson, I., Stalenheim, G., Rask, L., and Peterson, P. A., 1975, H-2 linked Ss protein is C4 component of complement, Nature 258: 243.PubMedGoogle Scholar
  223. 223.
    Day, N. K., Personal communication.Google Scholar
  224. 224.
    Day, N. K., Rubinstein, P., de Bracco, M., Moncada, B., Good, R. A., Hansen, J., Dupont, B., and Jersild, C., 1976, Hereditary Clr deficiency: lack of linkage to the HL-A region in two families (Abstract), J. Immunol., 116: 1731.Google Scholar
  225. 225.
    Fu, S. M., Stern, R., Kunkel, H. G., Dupont, B., Hansen, J. A., Day, N. K., Good, R. A., Jersild, C., and Fotino, M., 1975, Mixed lymphocyte culture determinants and C2 deficiency in four families, J. Exp. Med. 142: 495.PubMedGoogle Scholar
  226. 226.
    Gelfand, J. A., Sherins, R. J., Ailing, D. W., and Frank, M. M.. 1976, Effective prophylaxis of hereditary angioedema by an impeded androgen, Danazol (Abstract), Clin. Res. 24: 446A.Google Scholar
  227. 227.
    Grace, H. J., Brereton-Stiles, G. G., Vos, G. H., and Schonland, M., 1976, A family with partial and total deficiency of complement C3, S. Afr. Med. J. 50: 139.PubMedGoogle Scholar
  228. 228.
    Hauptmann, G., Sasportes, M., Tongio, M. M., Mayer, S., and Dausset, J., 1976, The localization of the Bf locus within the MHS region on chromosome no. 6, Tissue Antigens 7: 52.PubMedGoogle Scholar
  229. 229.
    Hobart, M. J., and Lachmann, P. J., Allotypes of complement components in man, Transplant. Rev., in press.Google Scholar
  230. 230.
    Jackson, C. G., Ochs, H. D., and Wedgwood, R. J., 1976, The interactions of the alternate and classical pathways of complement (C) in C deficiencies (Abstract), Federation Proc. 35: 655.Google Scholar
  231. 231.
    Lachmann, P. J., Grennan, D., Martin, A., and Demant, P., 1975. Identification of Ss protein as murine C4, Nature 258: 242.PubMedGoogle Scholar
  232. 232.
    Lim, D., Gewurz, A., Lint, T. F., Ghaze, M., Sepheri, B., and Gewurz, H., Absence of the sixth complement in a patient with repeated episodes of meningococcal meningitis, J. Pediatr., in press.Google Scholar
  233. 233.
    Mauff, G., Hummel, K., and Pulverer, G., 1975, Properdin Factor B (glycine-rich beta-glycoprotein or C3 proactivator)-polymorphism: genetic and biochemical aspects. First application to paternity cases, Z. Immunitaetsforsch. Exp. Ther. 150: 327.Google Scholar
  234. 234.
    Olaisen, B., Teisberg, P., Gedde-Dahl, T., Jr., and Thorsby, E., 1975, The Bf locus in the HLA region of chromosome 6: linkage and association studies. Humangenetik 30: 291.PubMedGoogle Scholar
  235. 235.
    Osofsky, S. G., Thompson, B. H., Lint, T. F., and Gewurz. H., Hereditary deficiency of the third component of complement in a child with fever, skin rash and arthralgias, and response to whole blood transfusion, submitted for publication.Google Scholar
  236. 236.
    Petersen, B. H., Graham, J. A., and Brooks, G. F., 1975, Human deficiency of the 8th component of complement: lack of bactericidal activity, Clin. Res. 23: 295A.Google Scholar
  237. 237.
    Raum, D., Schur, P. H., Hobart, M. J., and Alper, C. A., Unpublished observations.Google Scholar
  238. 238.
    Raum, D., Glass, D., Carpenter, C. B., Alper, C. A., and Schur, P. H., 1976, Linkage of Factor B, C2 deficiency, and HLA, and ordering of genes on chromosome six (Abstract), Clin. Res. 24: 335A.Google Scholar
  239. 239.
    Thompson, R. A., 1976, Proc. Br. Soc. Inzmunol., spring meeting, p. 33.Google Scholar
  240. 240.
    Jasin, H. E., Personal communication.Google Scholar
  241. 241.
    Rosenfeld, S. I., Kelly, M. E., and Leddy, J. P., 1976, Hereditary deficiency of the fifth component of complement in man. I. Clinical, immunochemical, and family studies, J. Clin. Invest. 57: 1626.PubMedCentralPubMedGoogle Scholar
  242. 242.
    Rosenfeld, S. I., Baum, J., Steigbigel, R. T., and Leddy, J. P., 1976, Hereditary deficiency of the fifth component of complement in man. II. Biological properties of CS-deficient human serum, J. Clin. Invest. 57: 1635.PubMedCentralPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1976

Authors and Affiliations

  • Chester A. Alper
    • 1
    • 2
  • Fred S. Rosen
    • 1
    • 2
  1. 1.Center for Blood Research Division of ImmunologyChildren’s Hospital Medical CenterUSA
  2. 2.Department of PediatricsHarvard Medical SchoolBostonUSA

Personalised recommendations