Skip to main content

Neutrophil and Complement Defects: Recent Advances

  • Chapter
Immunodeficiency and Disease

Part of the book series: Immunology and Medicine Series ((IMME,volume 8))

Abstract

Although a great deal has been learnt about the physiology of phagocytic cells and the complement system from in vitro studies, one cannot necessarily relate such findings to their physiological importance in vivo. It is only by the study of patients with well defined abnormalities of complement or phagocyte function that one can learn how these systems actually function in vivo. Of particular value have been the studies of patients with inherited deficiencies of single proteins. Patients with neutrophil and monocyte abnormalities frequently suffer from recurrent pyogenic infections, those with complement deficiencies develop recurrent infections and also have a high prevalence of immune complex disease. Although patients with inherited deficiencies are extremely rare, nevertheless they have taught us a great deal about the normal biology of both phagocytic cells and the complement system. Such patients represent ‘experiments of nature’ and the frequency with which such individuals are encountered is often inversely related to the impact they have upon our understanding of the biology of phagocytic cells, the complement system and their role in host defence.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 39.99
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 54.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. White, C. J. and Gallin, J. I. (1986). Phagocyte defects, Clin. Immunol. Immunopathol., 40 50–61

    PubMed  CAS  Google Scholar 

  2. Klebanoff, S. J. and Clark, R. A. (1978). In The Neutrophil. Ch. 10. (Amsterdam: North Holland)

    Google Scholar 

  3. Gallin, J. I. and Fauci, A. S. (eds.) (1983). Advances in Host Defence Mechanisms. Vol. 3. (New York: Raven Press)

    Google Scholar 

  4. Johnston, R. B. and Newman, S. L. (1977). Chronic granulomatous disease.Ped. Clin. N. Am., 24, 365–76

    CAS  Google Scholar 

  5. Quie, P. G., White, J. G., Holmes, B. and Good, R. A. (1967). In vitro bactericidal capacity of human polymorphonuclear leukocytes: diminished activity in chronic granulomatous disease of childhood. J. Clin. Invest., 46, 668–79

    PubMed  CAS  Google Scholar 

  6. Thompson, E. N. and Soothill, J. F. (1970). Chronic granulomatous disease: qualitative clinicopathological relationships. Arch. Dis. Child., 45, 24–32

    PubMed  CAS  Google Scholar 

  7. Segal, A. W. and Coade, S. (1978). Kinetics of oxygen consumption by phagocytosing human neutrophils. Biochem. Biophys. Res. Commun., 84, 611–17

    PubMed  CAS  Google Scholar 

  8. Baldridge, C. W. and Gerard, R. W. (1933). The extra respiration of phagocytosis. Am. J. Physiol., 103, 235–6

    CAS  Google Scholar 

  9. Sbarra, A. J. and Karnovsky, M. L. (1959). The biochemical basis of phagocytosis. I. Metabolic changes during the ingestion of particles by polymorphonuclear leukocytes. J. Biol. Chem., 234, 1355–62

    PubMed  CAS  Google Scholar 

  10. Selvaraj, R. J. and Sbarra, A. J. (1966). Relationship of glycolytic and oxidative metabolism to particle entry and destruction in phagocytosing cells. Nature (London), 211, 1272–6

    CAS  Google Scholar 

  11. Mandell, G. L. (1974). Bactericidal activity of aerobic and anaerobic polymorphonuclear neutrophils. Infect. Immunol., 9, 337–41

    CAS  Google Scholar 

  12. Holmes, B., Page, A. R. and Good, R. A. (1967). Studies of the metabolic activity of leukocytes from patients with a genetic abnormality of phagocyte function. J. Clin. Invest., 46, 1422–32

    PubMed  CAS  Google Scholar 

  13. Sternholm, R. and Manak, R. C. (1970). Carbohydrate metabolism in leukocytes. XIV. Regulation of pentose cycle activity and glycogen metabolism during phagocytosis. J. Reticuloendoth. Soc., 8, 550–60

    Google Scholar 

  14. Segal, A. W. and Jones, O. T. G. (1978). Novel cytochrome b system in phagocytic vacuoles from human granulocytes. Nature (London), 276, 515–17

    CAS  Google Scholar 

  15. Cross, A. R., Jones, O. T. G., Harper A. M. and Segal, A. W. (1981). Oxidation-reduction properties of the cytochrome b found in the plasma-membrane fraction of human neutrophils. A possible oxidase in the respiratory burst. Biochem. J., 194, 599–606

    PubMed  CAS  Google Scholar 

  16. Segal, A. W. and Jones, O. T. G. (1979). The subcellular distribution and some properties of the cytochrome b component of the microbicidal oxidase system of human neutrophils. Biochem. J., 182, 181–8

    PubMed  CAS  Google Scholar 

  17. Garcia, R. C. and Segal, A. W. (1984). Changes in the subcellular distribution of the cytochrome b-245 on stimulation of human neutrophils. Biochem. J., 219, 233–42

    PubMed  CAS  Google Scholar 

  18. Segal, A. W. and Jones, O. T. G. (1980). Rapid incorporation of the human neutrophil plasma membrane cytochrome b into phagocytic vacuoles. Biochem. Biophys. Res. Commun., 92, 710–15

    PubMed  CAS  Google Scholar 

  19. Cross, A. R., Higson, F. K., Jones, O. T. G., Harper, A. M. and Segal, A. W. (1982). The enzymic reduction and kinetics of oxidation of cytochrome b-245 of neutrophils. Biochem. J., 204, 479–85

    PubMed  CAS  Google Scholar 

  20. Cross, A. R., Parkinson, J. F. and Jones, O. T. G. (1985). Mechanism of the superoxide producing oxidase of neutrophils is necessary for the fast reduction of cytochrome b-245 bv NADPH. Biochem. J., 226, 881–4

    PubMed  CAS  Google Scholar 

  21. Segal, A. W. (1988). Absence of the two subunits of cytochrome b−245 from neutrophils in X-linked chronic granulomatous disease. Nature, In press

    Google Scholar 

  22. Harper, A. M., Chaplin, M. F. and Segal, A. W. (1985). Cytochrome b−245 from human neutrophils is a glycoprotein. Biochem. J., 227, 783–8

    PubMed  CAS  Google Scholar 

  23. Poole, R. K. (1983). Bacterial cytochrome oxidases. A structurally and functionally diverse group of electron-transfer proteins. Biochim. Biophys. Acta, 726, 205–43

    PubMed  CAS  Google Scholar 

  24. Cross, A. R., Jones, O. T. G., Garcia, R. and Segal, A. W. (1982). The association of FAD with the cytochrome b-245 of human neutrophils. Biochem. J., 208, 759–63

    PubMed  CAS  Google Scholar 

  25. Light, D. R., Walsh, C, O’Callaghan, A. M., Goetzl, E. J. and Tauber, A. I. (1981). Characteristics of the cofactor requirements for the superoxide-generating NADPH oxidase of human polymorphonuclear leukocytes. Biochemistry, 20, 1468–76

    PubMed  CAS  Google Scholar 

  26. Millard, J. A., Gerard, K. W. and Schneider, D. L. (1979). The isolation from rat peritoneal leukocytes of plasma membrane enriched in alkaline phosphatase and a b-type cytochrome. Biochem. Biophys. Res. Commun., 90, 312–19

    PubMed  CAS  Google Scholar 

  27. Crawford, D. R. and Schneider, D. L. (1982). Identification of ubiquinone 50 in human neutrophils and its role in microbicidal events. J. Biol. Chem., 257, 6662–8

    PubMed  CAS  Google Scholar 

  28. Cunningham, C. C, De Chatelet, L. R., Spach, P. I., Parce, J. W., Thomas, M. J., Lees, C. J. and Shirley, P. S. (1982). Identification and quantitation of electron transport components in human polymorphonuclear neutrophils. Biochim. Biophys. Acta, 682, 430–35

    PubMed  CAS  Google Scholar 

  29. Cross, A. R., Jones, O. T. G., Garcia, R. C. and Segal, A. W. (1983). The subcellular localization of ubiquinone in human neutrophils. Biochem. J., 216, 765–8

    PubMed  CAS  Google Scholar 

  30. Hitzig, W. H. and Seger, R. A. (1983). Chronic granulomatous disease, a heterogenous syndrome. Hum. Genet., 64, 207–15

    PubMed  CAS  Google Scholar 

  31. Hamers, M. N., de Boer, M., Meerhof, L. J., Weening, R. S. and Roos, D. (1988). Complementation in monocyte hybrids revealing genetic heterogeneity in chronic granulomatous disease. Nature (London), In press

    Google Scholar 

  32. Segal, A. W., Cross, A. R., Garcia, R. C, Borregaard, N., Valerius, N. H., Soothill, J. F. and Jones, O. T. G. (1983). Absence of cytochrome b-245 in chronic granulomatous disease. A multicentre European evaluation of its incidence and relevance. N. Engl. J. Med., 308, 245–51

    PubMed  CAS  Google Scholar 

  33. Ohno, Y., Buescher, E. S., Roberts, R., Metealf, J. A. and Gallin, J. (1986). Reevaultion of cytochrome b and flavin adenine dinucleotide in neutrophils from patients with chronic granulomatous disease and description of a family with probable autosomal recessive inheritance of cytochrome b deficiency. Blood, 67, 1132–8

    PubMed  CAS  Google Scholar 

  34. Franke, U., Ochs, H. D., de Martinville, B. et al. (1985). Minoe Xp 21 chromosome deletion in a malt associated with expression of Duchenne muscular dystropy, chronic gravulomators disease, retinits pigmentosa and McLeod syndrome. Am. J. Hum. Genet., 37, 250–67

    Google Scholar 

  35. Royer-Pokora, B. et al. (1986). Cloning the gene for an inherited human disorder — chronic granulomatous disease — on the basis of its chromosomal location. Nature, 322, 32–8

    PubMed  CAS  Google Scholar 

  36. Segal, A. W. and Jones, O. T. G. (1980). Absence of cytochrome b reduction in stimulated neutrophils from both female and male patients with chronic granulomatous disease. FEBS Letts., 110, 111–14

    CAS  Google Scholar 

  37. Gennaro, R., Florio, C. and Romeo, N. (1985). Activation of protein kinase C in neutrophil cytoplasts. Localization of protein substrates and possible relationship with stimulus-response coupling. FEBS Letts., 180, 185–90

    CAS  Google Scholar 

  38. Berridge, M. J. (1984). Inositol triphosphate and diacylglycerol as second messengers. Biochem. J., 220, 345–60

    PubMed  CAS  Google Scholar 

  39. Segal, A. W., Heyworth, P. G., Cockroft, S. and Barrowman, M. M. (1985). Stimulated neutrophils from patients with autosomal recessive chronic granulomatous disease fail to phosphorylate a M-44,000 kDa protein. Nature (London). 316, 547–9

    CAS  Google Scholar 

  40. Gray, G. R. et al. (1973). Neutrophil dysfunction, chronic granulomatous disease and nonspherocytic haemolytic anaemia caused by complete deficiency of glucose-6-phosphate dehydrogenase. Lancet, 2, 530–4

    PubMed  CAS  Google Scholar 

  41. Harvath, L. and Anderson, B. R. (1979). Defective initiation of oxidative metabolism in polymorphonuclear leukocytes. N. Engl. J. Med., 300, 1130–5

    PubMed  CAS  Google Scholar 

  42. Segal, A. W., Geisow, M., Garcia, R., Harper, A. and Miller, R. (1981). The respiratory burst of phagocytic cells is associated with a rise in vacuolar pH. Nature, 290, 406–9

    PubMed  CAS  Google Scholar 

  43. Babior, B. M., Kipnes, R. S. and Curnutte, J. T. (1973). The production by leukocytes of superoxide, a potential bactericidal agent.J. Clin. Invest., 52, 741–4

    PubMed  CAS  Google Scholar 

  44. Iyer, G. Y. N., Islam, D. M. F. and Quastel, J. H. (1961). Biochemical aspects of phagocytosis. Nature, 192, 535–41

    CAS  Google Scholar 

  45. Roos, D., Voetman, A. A. and Meerhof, L. J. (1983). Functional activity of enucleated human polymorphonuclear leukocytes. J. Cell Biol., 97, 368–77

    PubMed  CAS  Google Scholar 

  46. Ambruso, D. R. and Johnston, R. B. (1981). Lactoferrin enhances hydroxyl radical production by human neutrophils, neutrophil particulate fractions and an enzymic generating system.J. Clin. Invest., 67, 352

    PubMed  CAS  Google Scholar 

  47. Gutteridge, J. M., Paterson, S. K., Segal, A. W. and Halliwell, B. (1981). Inhibition of lipid peroxidation by the iron-binding protein lactoferrin. Biochem. J., 199, 259–61

    PubMed  CAS  Google Scholar 

  48. Klebanoff, S. J. (1975). Antimicrobial mechanisms in neutrophilic polymorphonuclear leukocytes. Semin. Hematol., 12, 117–42

    PubMed  CAS  Google Scholar 

  49. Winterbourn, C. C, Garcia, R. C. and Segal, A. W. (1985). Production of the superoxide adduct of myeloperoxidase (compound 111) by stimulated human neutrophils, and its reactivity with hydrogen peroxide and chloride. Biochem. J., 288, 583–92

    Google Scholar 

  50. Odeberg, H. and Olsson, I. (1975). Antibacterial activity of cationic proteins from human granulocytes. J. Clin. Invest., 56, 1118–24

    PubMed  CAS  Google Scholar 

  51. Barrett, A. J. (1969). Properties of lysosomal enzymes. In Dingle, J. T. and Fell, H. B. (eds.) Lysosomes in Biology and Pathology. Vol. 2, p. 244. (Amsterdam: North Holland)

    Google Scholar 

  52. Jacques, Y. V. and Bainton, D. F. (1978). Changes in pH within the phagocytic vacuoles of human neutrophils and monocytes. Lab. Invest., 39, 179–85

    PubMed  CAS  Google Scholar 

  53. Cech, P. and Lehrer, R. I. (1984). Phagolysosomal pH of human neutrophils. Blood, 63, 88–95

    PubMed  CAS  Google Scholar 

  54. Gabig, T. G., Lefker, B. A., Ossanna, P. J. and Weiss, S. J. (1984). Proton stoichiometry associated with human neutrophil respiratory-burst reactions.J. Biol. Chem., 259, 13166–71

    PubMed  CAS  Google Scholar 

  55. Segal, A. W., Harper, A. M., Garcia, R. C. and Merzbach, D. (1982). The action of cells from patients with chronic granulomatous disease on Staphylococcus aureus. J. Med. Microbiol., 15, 441–9

    PubMed  CAS  Google Scholar 

  56. Klebanoff, S. J. and Clark, R. A. (1978). The neutrophil. Function and clinical disorders, pp. 38–42. (Amsterdam, New York: North-Holland)

    Google Scholar 

  57. Landing, B. H. and Shirkey, H. S. (1957). A syndrome of recurrent infection and infiltration of viscera by pigmented lipid histiocytes. Pediatrics, 20, 431–8

    PubMed  CAS  Google Scholar 

  58. Newburger, P. E., Cohen, H. J., Rothchild, S. B. et al. (1979). Prenatal diagnosis of chronic granulomatous disease. N. Engl J. Med., 300, 181–3

    Google Scholar 

  59. Segal, A. W. and Peters, T. J. (1975). The nylon column dye test: a possible screening test of phagocytic function. Clin. Sci. Mol. Med., 49, 591–6

    PubMed  CAS  Google Scholar 

  60. Trush, M. A., Wilson, M. E. and Van Dyke, K. (1978). The generation of chemiluminescence (CL) by phagocytic cells. Meth. Enzymol., 57, 462

    CAS  Google Scholar 

  61. Babior, B. M. and Cohen, H. J. (1981). Measurement of neutrophil function: phagocytosis, degranulation, the respiratory burst and bacterial killing. In Cline, M. J. (ed.), Methods in Hematology, Vol. 3, pp. 1–38. (London: Churchill Livingstone)

    Google Scholar 

  62. Segal, A. W., Harper, A. M., Cross, A. R. and Jones, O. T. G. (1988). Purification and properties of cytochrome b-245. Meth. Enzymol., in press

    Google Scholar 

  63. Rausch, P. G., Pryzwansky K. B. and Spitznagel, J. K. (1978). Immunocytochemical identification of azurophilic and specific granule markers in giant granules of Chediak-Higashi neutrophils. N. Engl J. Med., 298, 693–8

    PubMed  CAS  Google Scholar 

  64. Pollard, H. B., Shindo, H., Creutz, C. E., Pazoles, C. J. and Cohen, J. S. (1979). Internal pH and state of ATP in adrenergic chromaffin granules determined by 31P nuclear magnetic resonance spectroscopy. J. Biol Chem., 254, 1170–7

    PubMed  CAS  Google Scholar 

  65. Hobart, M. J., Walport, M. J. and Lachmann, P. J. (1984). Complement polymorphism and disease. Clin. Immunol All, 4, 647–64

    Google Scholar 

  66. Lachmann, P. J. (1984). Inherited complement deficiencies. Philos. Trans. R. Soc. Lond. (Biol.), 306, 419–30

    CAS  Google Scholar 

  67. Ross, S. C. and Densen, P. (1984). Complement deficiency states and infection. Medicine, 62, 243–73

    Google Scholar 

  68. Donaldson, V. H., Hess, E. V. and McAdam, A. J. (1977). Lupus erythematosus-like syndrome in three unrelated women with hereditary angioneurotic edema. Ann. Intern. Med., 86, 312–13

    PubMed  CAS  Google Scholar 

  69. Brickman, C. M., Tsokos, G. C, Balow, J. E., Lawley, T. J., Santaella, M., Hammer, C. H. and Frank, M. M. (1986). Immunoregulatory disorders associated with hereditary angioedema. I. Clinical manifestations of autoimmune disease. J. Allergy Clin. Immunol., 77, 749–57.

    PubMed  CAS  Google Scholar 

  70. Heidelberger, M. (1941). Quantitative chemical studies on complement or alexin. J. Exp. Med., 73, 681–94

    PubMed  CAS  Google Scholar 

  71. Schifferli, J. A., Ng, Y. C. and Peters, D. K. (1986). The role of complement and its receptor in the elimination of immune complexes. N. Engl. J. Med., 315, 488–95

    PubMed  CAS  Google Scholar 

  72. Lachmann, P. J. and Walport, M. J. (1988). Deficiency of the effector mechanisms of the immune response and autoimmunity. In Whelan, J. (ed.) Autoimmunity and Autoimmune Disease (Ciba Foundation Symposium 129). (Chichester: Wiley) in press

    Google Scholar 

  73. Joseph, B. S. and Oldstone, M. B. A. (1974). Antibody-induced redistribution of measles virus antigen on the cell surface. J. Immunol., 113, 1205–11

    PubMed  CAS  Google Scholar 

  74. Gorman, N. T. and Lachmann, P. J. (1982). In vitro modulation of viral cell surface glycoproteins by anti-viral antibody in the presence of complement. Clin. Exp. Immunol., 50,507–14

    PubMed  CAS  Google Scholar 

  75. Rittner, C, Meier, E. M. M., Stradmann, B., Giles, C. M., Kochling, R., Mollenhauer, E. and Kretch, H. W. (1984). Partial C4 deficiency in subacute sclerosing panencephalitis. Immunogenetics, 20, 407–15

    PubMed  CAS  Google Scholar 

  76. Mauff, G., Alper, C. A., Awdeh, Z., Batchelor, J. R., Bertrams, J., Bruun-Peterson, G., Dawkins, R. L., Demant, P., Edwards, J., Grosse-Wilde, H., Hauptmann, G., Klouda, P., Lamm, L., Mollenhauer, E., Neri, C, Olaisen, B., O’Neill, G., Rittner, C, Roos, M. H., Skanes, V., Teisberg, P. and Wells, L. (1983). Statement on the nomenclature of human C4 allotypes. Immunobiology, 164, 184–91

    PubMed  CAS  Google Scholar 

  77. Yu, C. T., Belt, K. T., Giles, C. M., Campbell, R. D. and Porter, R. R. (1986). Structural basis of the polymorphism of human complement components C4A and C4B: gene size, reactivity and antigenicity. EMBO J., 5, 2873–81

    PubMed  CAS  Google Scholar 

  78. Law, S. K. A., Dodds, A. W. and Porter, R. R. (1984). A comparison of the properties of two classes, C4A and C4B, of the complement component C4. EMBO J., 3, 1819–23

    PubMed  CAS  Google Scholar 

  79. Isenman, D. and Young, J. R. (1984). The molecular basis for the differences in immune hemolysis activity of the Chido and Rodgers isotypes of human complement component C4. J. Immunol., 132, 3019–27

    PubMed  CAS  Google Scholar 

  80. Hauptman, G., Goetz, J., Uring-Lambert, B. and Grosshans, E. (1986). Component deficiencies. 2. The fourth component. Progr. Allergy, 39, 232–49

    Google Scholar 

  81. Alper, C. A., Raum, D., Karp, C, Awdeh, Z. L. and Yunis, E. J. (1983). Serum complement supergenes of the major histocompatibility complex in man (complotypes). Vox Sang., 45, 62–7

    PubMed  CAS  Google Scholar 

  82. Glass, D., Raum, D., Gibson, D., Stillman, J. S. and Schur, P. M. (1976). Inherited deficiency of the second component of complement. J. Clin. Invest., 58, 853–61

    PubMed  CAS  Google Scholar 

  83. Walport, M. J., Fielder, A. H. L. and Batchelor, J. R. (1984). Genetics of systemic lupus erythematosus. In Panayi, G. and David, C. (eds.) Clinical Immunology. I. Immunogenetics. pp. 157–76. (Sevenoaks, Kent: Butterworth)

    Google Scholar 

  84. Fielder, A. H. L., Walport, M. J., Batchelor, J. R., Rynes, R. I., Black, C. M., Dodi, I. A. and Hughes, G. R. V. (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., 266, 425–8

    Google Scholar 

  85. Schifferli, J. A. and Peters, D. K. (1983). Complement, the immune-complex lattice and the pathophysiology of complement-deficiency syndrome. Lancet, 2, 957–9

    PubMed  CAS  Google Scholar 

  86. Lachmann, P. J. (1988). Deficiencies of Factor I and Factor H. In Rother, K. and Till, G. (eds.) The Complement System. (Springer-Verlag) In press

    Google Scholar 

  87. Sjoholm, A. G., Braconier, J.-H. and Soderstrom, C. (1982). Properdin deficiency in a family with fulminant meningococcal infections. Clin. Exp. Immunol, 50, 291–7

    PubMed  CAS  Google Scholar 

  88. Mauff, G., Federman, G. and Hauptmann, G. (1980). A haemolytically inactive gene product of factor B. Immunobiology, 158, 96–100

    PubMed  CAS  Google Scholar 

  89. Hannecke, C, Kluin-Nelemans, van Velzen-Blad, H., van Helden, H. P. T. and Daha, M. R. (1984). Functional deficiency of complement factor D in a monozygous twin. Clin. Exp. Immunol., 58, 724–30

    Google Scholar 

  90. Merino, J., Rodriguez-Valverde, V., Lamelas, J. A., Riestra, J. L. and Casanueva, B. (1983). Prevalence of deficits of complement components in patients with recurrent meningococcal infections. J. Infect. Dis., 148, 331

    PubMed  CAS  Google Scholar 

  91. Ellison, R. T., Kohler, P. F., Curd, J. G., Judson, F. N. and Relier, L. B. (1983). Prevalence of congenital or acquired complement deficiency in patients with sporadic meningococcal disease. N. Engl. J. Med., 308, 913–16

    PubMed  Google Scholar 

  92. Inai, S., Kitamura, H., Hiramatsu, S. and Nagaki, K. (1979). Deficiency of the ninth component of complement in man. J. Clin. Lab. Immunol., 2, 85–7

    PubMed  CAS  Google Scholar 

  93. Fine, D. P., Gewurz, H., Griffiss, M. and Lint, T. F. (1983). Meningococcal meningitis in a woman with inherited deficiency of the ninth component of complement. Clin. Immunol Immunopathol., 28, 413–17

    PubMed  CAS  Google Scholar 

  94. Rosenfield, S. I., Kelly, M. E. and Leddy, J. P. (1976). Hereditary deficiency of the fifth component in man. J. Clin. Invest., 57, 1626–43

    Google Scholar 

  95. Jasin, H. E. (1977). Absence of the eighth component of complement in association with systemic lupus erythematosus-like disease. J. Clin. Invest., 60, 709–15

    PubMed  CAS  Google Scholar 

  96. Zeitz, H. J., Miller, G. W., Lint, T. F., Ali, M. A. and Gewurz, H. (1981). Deficiency of C7 with systemic lupus erythematosus. Solubilization of immune complexes in complement deficient sera. Arthritis Rheum., 24, 87–93

    PubMed  CAS  Google Scholar 

  97. Pickering, R. J., Rynes, R. I., Locascio, N., Monahan, J. B. and Sodetz, J. M. (1982). Identification of the alpha-gamma subunit of the eighth component of complement (C8) in a patient with systemic lupus erythematosus and absent C8 activity: patients and family studies. Clin. Immunol Immunopathol., 23, 323–4

    PubMed  CAS  Google Scholar 

  98. Ross, G. D. and Medof, M. E. (1985). Membrane complement receptors specific for bound fragments of C3. Adv. Immunol., 37, 217–67

    PubMed  CAS  Google Scholar 

  99. Wilson, J. G., Wong, W. W., Schur, P. H. and Fearon, D. T. (1982). Mode of inheritance of decreased C3b receptors on erythrocytes of patients with systemic lupus erythematosus. N. Engl. J. Med., 307, 981–6

    PubMed  CAS  Google Scholar 

  100. Walport, M. J., Ross, G. D., Mackworth-Young, C, Watson, J. V., Hogg, N. and Lachmann, P. J. (1985). Family studies of erythrocyte complement receptor type I levels: reduced levels in patients with SLE are acquired, not inherited. Clin. Exp. Immunol., 59, 547–54

    PubMed  CAS  Google Scholar 

  101. Wilson, J. G., Murphy, E. E., Wong, W. W., Klickstein, L. B., Weis, J. M. and Fearon, D. T. (1986). Identification of a restriction fragment length polymorphism by a CR1 cDNA that correlates with the number of CR1 on erythrocytes. J. Exp. Med., 315, 488–95

    Google Scholar 

  102. Melchers, F. A., Erdei, T. and Dierich, M. P. (1985). Growth control of activated, synchronized murine B cells by the C3d fragment of human complement. Nature, 317, 264–7

    PubMed  CAS  Google Scholar 

  103. Bottger, E. C, Hoffmann, T., Hadding, U. and Bitter-Suermann, D. (1985) Influence of genetically inherited complement deficiencies on humoral immune response in guinea pigs. J. Immunol., 135, 4100–7

    PubMed  CAS  Google Scholar 

  104. Fingeroth, J. D., Weiss, J. J., Tedder, T. F., Strominger, J. L., Biro, P. A. and Fearon, D. T. (1984). Epstein-Barr virus receptor of human B lymphocytes is the C3d receptor, CR2. Proc. Natl. Acad. Sci. (USA), 81, 4510–14

    CAS  Google Scholar 

  105. Nemerow, G., Wolfert, R., McNaughton, M. E. and Cooper, N. R. (1985). Identification and characterization of the Epstein-Barr virus receptor on human B lymphocytes and its relationship to the C3d complement receptor (CR2). J. Virol., 55, 347–51

    PubMed  CAS  Google Scholar 

  106. Young, L. S., Clark, D., Sixbey, J. W. and Rickinson, A. B. (1986). Epstein-Barr virus receptors on human pharyngeal epithelia. Lancet, 1, 240–2

    PubMed  CAS  Google Scholar 

  107. Sixbey, J. W., Davis, D. S., Young, L. S., Hutt-Fletcher, L., Tedder, T. F. and Rickinson, A. B. (1986). Human epithelial cell expression of an Epstein-Barr virus receptor. Clin. Res., 34, 533A (abstract)

    Google Scholar 

  108. Weis, J. J., Fearon, D. T., Klickstein, L. B., Wong, W. W., Richards, S. A., De Bruyn Kops, A., Smith, H. A. and Weis, J. M. (1986). Identification of a partial cDNA clone for the C3d/Epstein-Barr virus receptor of human B lymphocytes: homology with the receptor for fragments C3b and C4b of the third and fourth components of complement. Proc. Natl. Acad. Sci. (USA), 83, 5639–43

    PubMed  Google Scholar 

  109. Holers, V. M., Cole, J. L., Lublin, D. M., Seya, T. and Atkinson, J. P. (1985). Human C3b- and C4b-regulatory proteins: a new multi-gene family. Immunol. Today, 6, 188–92

    CAS  Google Scholar 

  110. Yoon, S. M. and Fearon, D. T. (1985). Characterization of a soluble form of the C3b/C4b receptor (CR1) in human plasma. J. Immunol, 134, 3332–8

    PubMed  CAS  Google Scholar 

  111. Moldenhauer, F., David, J., Fielder, A. H. L., Lachmann, P. J. and Walport, M. J. (1988). Inherited deficiency of erythrocyte complement receptor type 1 does not cause disease susceptibility to SLE. Arthritis Rheum., in press

    Google Scholar 

  112. Wong, W. W., Kennedy, C. A., Bonaccio, E. T., Wilson, J. G., Klickstein, L. B., Weis, J. H. and Fearon, D. T. (1986). Analysis of multiple restriction fragment length polymorphisms of the gene for the human complement receptor type 1. J. Exp. Med., 164, 1531–46

    PubMed  CAS  Google Scholar 

  113. Ross, G. D., Lambris, J. D., Cain, J. A. and Newman, S. L. (1982). Generation of three different fragments of bound C3 with purified factor I or serum. I. Requirements for factor H vs CR1 cofactor activity. J. Immunol., 129, 2051–60

    PubMed  CAS  Google Scholar 

  114. Medof, M. E., Iida, K., Mold, C. and Nussenzweig, V. (1982). Unique role of the complement receptor CR1 in the degradation of C3b associated with immune complexes. J. Exp. Med., 156, 1739–54

    PubMed  CAS  Google Scholar 

  115. Medicus, R. G., Melamed, J. and Arnaout, M. A. (1983). Role of human factor I and C3b receptor in the cleavage of surface-bound C3bi molecules. Eur. J. Immunol, 13, 465–70

    PubMed  CAS  Google Scholar 

  116. Medof, M. E. and Oger, J. J.-F. (1982). Competition for immune complexes by red cells in human blood. J. Clin. Lab. Immunol., 7, 7–13

    PubMed  CAS  Google Scholar 

  117. Cornacoff, J. B., Hebert, L. A., Smead, W. L., Van Aman, M. E., Birmingham, D. J. and Waxman, F. J. (1983). Primate erythrocyte-immune complex-clearing mechanism. J. Clin. Invest., 71, 236–47

    PubMed  CAS  Google Scholar 

  118. Waxman, F. J., Herbert, L. A., Cornacoff, J. B., Van Aman, M. E., Smead, W. L., Kraut, E. M., Birmingham, D. J. and Taquiam, J. M. (1984). Complement depletion accelerates the clearance of immune complexes from the circulation of primates. J. Clin. Invest, 74, 1329–40

    PubMed  CAS  Google Scholar 

  119. Waxman, F. J., Herbert, L. A., Cosio, F. G., Smead, W. L., Van Aman, M. E., Taquiam, J. M. and Birmingham, D. J. (1986). Differential binding of immunoglobulin A and immunoglobulin Gl immune complexes to primate erythrocytes in vivo. J. Clin. Invest., 11, 82–9

    Google Scholar 

  120. Schifferli, J. A., Ng, Y.C., Estreichee, J., Walport, M. J. (1988). The clearance of tetanus toxoid/anti-tetanus toxoid immune complexes from the circulation of humans. J. Immunol., 140, No.3

    Google Scholar 

  121. Miyakawa, Y., Yamada, A., Kosaka, K., Tsuda, F., Kosugi, E. and Mayumi, M. (1981). Defective immune-adherence (C3b) receptor on erythrocytes from patients with systemic lupus erythematosus. Lancet, 2, 493–7

    PubMed  CAS  Google Scholar 

  122. Iida, K., Mornaghi, R. and Nussenzweig, V. (1982). Complement receptor (CR1) deficiency in erythrocytes from patients with systemic lupus erythematosus. J. Exp. Med., 155, 1427–38

    PubMed  CAS  Google Scholar 

  123. Minota, S., Terai, C., Nojima, Y., Takano, K., Takai, E., Miyakawa, Y. and Takaku, F. (1984). Low C3b receptor reactivity on erythrocytes from patients with systemic lupus erythematosus detected by immune adherence hemagglutination and radioimmunoassays with monoclonal antibody. Arthritis Rheum., 21, 1329–35

    Google Scholar 

  124. Ross, G. D., Yount, W. J„ Walport, M. J., Winfield, J. B., Parker, C. J., Fuller, C. R., Taylor, R. P., Myones, B. L. and Lachmann, P. J. (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, 2005–13

    PubMed  CAS  Google Scholar 

  125. Walport, M. J., Ng, Y. C, Lachmann, P. J. (1987). Erythrocytes transfused into patients with SLE and haemolytic avaeuia lose complement receptor type 1 from their cell surface. Clin. Exp. Immunol., 59, 547–54

    Google Scholar 

  126. Pangburn, M. K., Schreiber, R. D., Trombold, J. S. and Muller-Eberhard, H. J. (1983). Paroxysmal nocturnal hemoglobinuria: deficiency of factor H-like function of the abnormal erythrocytes. J. Exp. Med., 157, 1971–80

    PubMed  CAS  Google Scholar 

  127. Tausk, F. A., McCutchan, J. A., Spechko, P., Schrieber, R. D. and Gigli, I. (1986). Altered erythrocyte C3b receptor expression, immune complexes, and complement activation in homosexual men in varying risk groups for acquired immune deficiency syndrome. J. Clin. Invest., 78, 977–82

    PubMed  CAS  Google Scholar 

  128. Inada, Y., Kamiyama, M., Kanemitsu, T., Hyman, C. L. and Clark, W. S. (1982). Studies on immune adherence receptor (C3b) receptor activity of human erythrocytes: relation between receptor activity and presence of immune complexes in serum. Clin. Exp. Immunol., 50, 189–97

    PubMed  CAS  Google Scholar 

  129. Marlin, S. D., Morton, C. C, Anderson, D. C. and Springer, T. A. (1986). LFA-1 immunodeficiency disease. Definition of the genetic defect and chromosomal mapping of A and B subunits of the lymphocyte function-associated antigen (LFA-1) by complementation in hybrid cells. J. Exp. Med., 164, 855–67

    PubMed  CAS  Google Scholar 

  130. Springer, T. A., Telpow, D. B. and Dreyer, W. J. (1985). Sequence homology of the LFA-1 and Mac-1 leukocyte adhesion glycoproteins and unexpected relation to leukocyte interferon. Nature, 314, 540–2

    PubMed  CAS  Google Scholar 

  131. Suzuki, S., Argraves, W. S., Pytela, R., Arai, H., Krusius, T., Pierschbacher, M. D. and Ruoslahti, E. (1986). cDNA and amino acid sequences of the cell adhesion protein receptor recognising vitronectin reveal a transmembrane domain and homologies with other adhesion protein receptors. Proc. Natl. Acad. Sci. USA, 83, 8614–18

    PubMed  Google Scholar 

  132. Cosgrove, L. J., Sandrin, M. S., Rajasekariah, P. and McKenzie, I. F. C. (1986). A genomic clone encoding an alpha chain of the OKM 1, LFA-1 and platelet glycoprotein IIb-IIIa molecules. Proc. Natl. Acad. Sci. USA, 83, 752–6

    PubMed  CAS  Google Scholar 

  133. Sastre, L., Roman, J. M., Telow, D. B., Dreyer, W. J., Gee, C. E., Larson, R. S., Roberts, T. M. and Springer, T. A. (1986). A partial genomic DNA clone for the subunit of a mouse complement receptor type 3 and cellular adhesion molecule Mac-1. Proc. Natl. Acad. Sci. USA, 83, 5644–8

    PubMed  CAS  Google Scholar 

  134. Ross, G. D., Cain, J. A. and Lachmann, P. J. (1985). Membrane complement receptor type three (CR3) has lectin-like properties analogous to bovine conglutinin and functions as a receptor for zymosan and rabbit erythrocytes as well as a receptor for iC3b. J. Immunol., 134, 3307–15

    PubMed  CAS  Google Scholar 

  135. Wright, S. D. and Jong, M. T. C. (1986). Adhesion-promoting receptors on human macrophages recognize Escherichia coli by binding to lipopolysaccharide. J. Exp. Med., 164, 1876–88

    PubMed  CAS  Google Scholar 

  136. Lachmann, P. J., Elias, D. E. and Moffett, A. (1972). Conglutinin and immunecon-glutinins. In Ingram, D. G. (ed.) Biological Activities of Complement, p. 202 (Basel: Karger)

    Google Scholar 

  137. Ross, G. D., Thompson, R. A., Walport, M. J., Springer, T. A., Watson, J. V., Ward, R. H. R., Lida, J., Newman, S. L., Harrison, R. A. and Lachmann, P. J. (1985). Characterization of patients with an increased susceptibility to bacterial infections and a genetic deficiency of leukocyte membrane complement antigen LFA-1. Blood, 66, 882–90

    PubMed  CAS  Google Scholar 

  138. Micklem, K. J. and Sim, R. B. (1985). Isolation of complement-fragment-iC3b-binding-proteins by affinity chromatography. Biochem. J., 231, 233–6

    PubMed  CAS  Google Scholar 

  139. Anderson, D. C, Miller, L. J., Schmalstieg, F. C., Rothlein, R. and Springer, T. A. (1986). Contributions of the Mac-1 glycoprotein family to adherence-dependent granulocyte functions: structure-function assessments employing subunit-specific monoclonal antibodies. J. Immunol., 137, 15–28

    PubMed  CAS  Google Scholar 

  140. Mentzer, S. J., Gromkowski, S. H., Krensky, A. M., Burakoff, S. J. and Martz, E. (1985). LFA-1 membrane molecule in the regulation of homotypic adhesions of human B lymphocytes. J. Immunol., 135, 9–11

    PubMed  CAS  Google Scholar 

  141. Patarroyo, M., Beatty, P. G., Fabre, J. W. and Gahmberg, C. G. (1985). Identification of a cell surface protein complex mediating phorbol ester-induced adhesion (binding) among human mononuclear leukocytes. Scand. J. Immunol., 22, 171

    PubMed  CAS  Google Scholar 

  142. Rothlein, R. and Springer, T. A. (1986). The requirement for lymphocyte function-associated antigen 1 in homotypic leukocyte adhesion stimulated by phorbol ester. J. Exp. Med., 163, 1132–49

    PubMed  CAS  Google Scholar 

  143. Miller, L. J., Schwarting, R. and Springer, T. A. (1986). Regulated expression of the Mac-1, LFA-1, p 150,95 glycoprotein family during leukocyte differentiation. J. Immunol., 137, 2891–900

    PubMed  CAS  Google Scholar 

  144. Kay, A. B., Glass, J. and Salter, McG. (1979). Leuco-attractants enhance complement receptors on human phagocytic cells. Clin. Exp. Immunol., 38, 294–9

    PubMed  CAS  Google Scholar 

  145. Fearon, D. T. and Collins, L. A. (1983). Increased expression of C3b receptors on polymorphonuclear leukocytes induced by chemotactic factors and purification procedures. J. Immunol., 130, 370–5

    PubMed  CAS  Google Scholar 

  146. Berger, M., O’Shea, J., Cross, A. S., Folks, T. M., Chused, T. M., Brown, E. J. and Frank, M. M. (1984). Human neutrophils increase expression of C3bi as well as C3b receptors upon activation. J. Clin. Invest., 74, 1566–77

    PubMed  CAS  Google Scholar 

  147. Richerson, H. B., Walsh, G. M., Walport, M. J., Mogbel, R. and Kay, A. B. (1985). Enchancement of human neutrophil complement receptors: a comparison of the rosette technique with the uptake of radio-labelled anti-CRl monoclonal antibody. Clin. Exp. Immunol., 62, 442–8

    PubMed  CAS  Google Scholar 

  148. Todd, III R. F., Arnaout A., Rosin, R. E., Crowley, C. A., Peters, W. A. and Babior, B. M. (1984). Subcellular localization of the large subunit of Mol (Mol alpha; formerly gp 110) a surface glycoprotein associated with neutrophil adhesion. J. Clin. Invest., 74, 1280–90

    PubMed  CAS  Google Scholar 

  149. Arnaout, M. A., Spits, H., Terhorst, C., Pitt, J. and Todd, III R. F. (1984). Deficiency of a leukocyte surface glycoprotein (LFA-1) on two patients with Mol deficiency. Effects of cell activations on Mol/LFA-1 surface expression in normal and deficient leukocytes. J. Clin. Invest., 74, 1291–300

    PubMed  CAS  Google Scholar 

  150. O’Shea, J.J., Brown, E. J., Seligmann, B. E., Metcalf, J. A., Frank, M. M. and Gallin, J. I. (1985). Evidence for distinct intracellular pools of receptors for C3b and C3bi in human neutrophils. J. Immunol, 134, 2580–7

    PubMed  Google Scholar 

  151. Springer, T. A., Miller, L. J. and Anderson, D. C. (1986). p 150, 95 The third member of the MAC-1, LFA-1 human leukocyte adhesion glycoprotein family. J. Immunol., 136, 240–5

    PubMed  Google Scholar 

  152. Wright, S. D. and Meyer, B. C. (1986). Phorbol esters cause sequential activation and deactivation of complement receptors on polymorphonuclear leukocytes. J. Immunol., 136, 1759–64

    PubMed  CAS  Google Scholar 

  153. Wright, S. D., Detmers, P. A., Jong, M. T. C. and Meyer, B. C. (1986). Interferon-gamma depresses binding of ligand by C3b and C3bi receptors on cultured human monocytes, an effect reversed by fibronectin. J. Exp. Med., 163, 1245–59

    PubMed  CAS  Google Scholar 

  154. Bohnsack, J. F., Kleinman, H. K., Takahashi, T., O’Shea, J. J. and Brown, E. J. (1985). Connective tissue proteins and phagocytic cell function: laminin enhances complement and Fc-mediated phagocytosis by cultured human macrophages. J. Exp. Med., 161, 912–23

    PubMed  CAS  Google Scholar 

  155. Wright, S. D., Craigmyle, L. S. and Silverstein, S. C. (1983). Fibronectin and serum amyloid P component stimulate C3b- and C3bi-mediated phagocytosis in cultured human monocytes. J. Exp. Med., 158, 1338–43

    PubMed  CAS  Google Scholar 

  156. Wright, S. D. and Silverstein, S. C. (1982). Tumor-promoting phorbol esters stimulate C3b and C3b’ receptor-mediated phagocytosis in cultured human monocytes. J. Exp. Med., 156, 1149–64

    PubMed  CAS  Google Scholar 

  157. Gallin, J. I. (1985). Leukocyte adherence-related glycoproteins LFA-1, Mol and p 150, 95; a new group of monoclonal antibodies, a new disease and a possible opportunity to understand the molecular basis of leukocyte adherence. J. Infect. Dis., 152, 661–3

    PubMed  CAS  Google Scholar 

  158. Hayward, A. R., Leonard, J., Wood, C. B. S., Harvey, B. A. M., Greenwood, M. C. and Soothill, J. F. (1979). Delayed separation of the umbilical cord, widespread infections and defective neutrophil mobility. Lancet, 1, 1099–111

    PubMed  CAS  Google Scholar 

  159. Bowen, J., Ochs, H. D. and Wedgwood, R. J. (1979). Chemotaxis and umbilical separation. Lancet, 2, 302

    PubMed  CAS  Google Scholar 

  160. Crowley, C. A., Curnutte, J. T., Rosin, R. E., Andrew-Schwartz, J., Gallin, J. I., Klempner, M., Snyderman, R., Southwick, F. S., Stossel, T. P. and Babior, B. M. (1980). An inherited abnormality of neutrophil adhesion: its genetic transmission and its association with a missing protein. N. Engl. J. Med., 302, 1163–8

    PubMed  CAS  Google Scholar 

  161. Abramson, J. S., Mills, E. L., Sawyer, M. K., Regelmann, W. R., Nelson, J. D. and Quie, P. G. (1981). Recurrent infections and delayed separation of the umbilical cord in an infant with abnormal phagocytic cell locomotion and oxidative response during particle phagocytosis. J. Pediatr., 99, 887–94

    PubMed  CAS  Google Scholar 

  162. Bissenden, J. G., Haeney, M. R., Tarlow, M. J. and Thompson, R. A. (1981). Delayed separation of the umbilical cord, severe widespread infections and immunodeficiency. Arch. Dis. Child., 56, 397–9

    PubMed  CAS  Google Scholar 

  163. Buchanan, M. R., Crowley, C. A., Rosin, R. E., Gimbrone, Jnr M. A. and Babior, B. M. (1982). Studies on the interaction between GP-180-deficient neutrophils and vascular endothelium. Blood, 60, 160–5

    PubMed  CAS  Google Scholar 

  164. Fischer, A., Trung, P. H., Descamps-Latscha, B., Lisowska Grospierre, B., Gerota, L, Pere, N., Scheinmetzier, C., Durandy, A., Virelixier, J. L. and Griscelli, C. (1983). Bone-marrow transplantation for inborn error of phagocytic cells associated with defective adherence, chemotaxis and oxidative response during opsonized particle phagocytosis. Lancet, 2, 473–6

    PubMed  CAS  Google Scholar 

  165. Kobayashi, K. J., Fujita, K., Okino, F. and Kajii, T. (1984). An abnomality of neutrophil adhesion: autosomal recessive inheritance associated with missing neutrophil glycoproteins. Pediatrics, 73, 606–10

    PubMed  CAS  Google Scholar 

  166. Davies, E. G., Isaacs, D. and Levinsky, R. J. (1982). Defective immune interferon production and natural killer activity associated with poor neutrophil mobility and delayed umbilical cord separation. Clin. Exp. Immunol., 50, 454–60

    PubMed  CAS  Google Scholar 

  167. Bowen, T. J., Ochs, H. D., Altman, L. C., Price, T. H., Van Epps, D. P., Brautigan, D. L., Rosin, R. E., Perkins, W. D., Babior, B. N. M., Klebanoff, S. J. and Wedgwood, R. J. (1982). Severe recurrent bacterial infections associated with defective adherence and chemotaxis in two patients with neutrophils deficient in a cell-associated glycoprotein. J. Pediatr., 101, 932–9

    PubMed  CAS  Google Scholar 

  168. Beatty, P. G., Ochs, H. D., Harlan, J. M., Price, T. H., Rosen, H., Taylor, R. F., Hansen, J. A. and Klebanoff, S. J. (1984). Absence of monoclonal antibody-defined protein complex in a boy with abnormal leukocyte function. Lancet, 1, 535–7

    PubMed  CAS  Google Scholar 

  169. Arnaout, M. A., Pitt, J., Cohen, H. J., Melamed, J., Rosen, F. S. and Colten, H. R. (1982). Deficiency of a granulocyte-membrane glycoprotein (gpl50) in a boy with recurrent bacterial infections. N. Engl. J. Med., 306, 696–9

    Google Scholar 

  170. Anderson, D. C., Schmalsteig, F. C., Finegold, M. H. J., Hughes, B. J., Rothlein, R., Miller, L. J., Kohl, S., Tosi, M. F., Jacobs, R. L., Waldrop, T. C., Goldman, A. S., Shearer, W. T. and Springer, T. A. (1985). The severe and moderate phenotypes of hereditary Mac-1 LFA-1 deficiency: their quantitative definition and relation to leukocyte dysfunction and clinical features. J. Infect. Dis., 152, 668–89

    PubMed  CAS  Google Scholar 

  171. Anderson, D. C., Schmalsteig, F. C., Kohl, S., Arnaout, M. A., Tosi, M. F., Dana, N., Buffone, G. J., Hughes, B. J., Brinkley, B. R., Dickey, W. D., Abramson, J. S., Springer, T., Boxer, L. A., Hollers, J. M. and Smith, C. W. (1984). Abnormalities of polymorphonuclear leukocyte function associated with a heritable deficiency of high molecular weight surface glycoproteins (GP138): common relationship to diminished cell adherence. J. Clin. Invest., 74, 536–51

    PubMed  CAS  Google Scholar 

  172. Thompson, R. A., Candy, D. C. A. and McNeish, A. I. (1984). Familial defect of polymorph neutrophil phagocytosis associated with absence of a surface glycoprotein antigen (OKM1). Clin. Exp. Immunol., 58, 229–36

    PubMed  CAS  Google Scholar 

  173. Dana, N., Todd, R. F. III, Pitt, J., Springer, T. A. and Arnaout, M. A. (1984). Deficiency of a surface membrane glycoprotein (Mol) in man. J. Clin. Invest., 73, 153–9

    PubMed  CAS  Google Scholar 

  174. Sanchez-Madrid, F., Nagy, J. A., Robbins, E., Simon, P. and Springer, T. A. (1983). A human leukocyte differentiation antigen family with distinct alpha subunits and a common beta subunit: the lymphocyte-function associated antigen (LFA-1), the C3bi complement receptor (OKM1/Mac-1) and the p 150,95 molecule. J. Exp. Med., 158, 1785–98

    PubMed  CAS  Google Scholar 

  175. Springer, T. A., Thompson, W. S., Miller, L. J., Schmalstieg, F. C. and Anderson, D. C. (1984). Inherited deficiency of the Mac-1, LFA-1, p 150,95 glycoprotein family and its molecular basis. J. Exp. Med., 160, 1901–18

    PubMed  CAS  Google Scholar 

  176. Fischer, A., Durandy, A., Sterkers, G. and Griscelli, C. (1986). Role of the LFA-1 molecule in cellular interactions required for antibody production in humans. J. Immunol., 136, 3198–202

    PubMed  CAS  Google Scholar 

  177. Kohl, S., Springer, T. A., Schmalstieg, F. C., Loo, L. S. and Anderson, D. C. (1984). Defective natural killer cytotoxicity and polymorphonuclear leukocyte antibody-dependent cellular cytotoxicity in patients with LFA-1/OKM-1 deficiency. J. Immunol., 133, 2972–8

    PubMed  CAS  Google Scholar 

  178. Harlan, J. M., Killen P. D., Senecal, F. M., Schwartz, B. R., Yee, E. K., Taylor, R. F., Beatty, P. G., Price, T. H. and Ochs, H. D. (1985). The role of neutrophil membrane glycoprotein GP-150 in neutrophil adherence to endothelium in vitro. Blood, 66, 167–78

    PubMed  CAS  Google Scholar 

  179. Ross, G. D., Newman, S. L., Lambris, J. D., Devery-Pocius, J., Cain, J. A. and Lachmann, P. J. (1983). Generation of three different fragments of bound C3 with purified factor I or serum. II. Location of binding sites in the C3 fragments for factors B and H, complement receptors, and bovine conglutinin. J. Exp. Med., 158, 334–52

    PubMed  CAS  Google Scholar 

Download references

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 1988 Kluwer Academic Publishers

About this chapter

Cite this chapter

Segal, A., Walport, M. (1988). Neutrophil and Complement Defects: Recent Advances. In: Webster, A.D.B. (eds) Immunodeficiency and Disease. Immunology and Medicine Series, vol 8. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-1275-5_8

Download citation

  • DOI: https://doi.org/10.1007/978-94-009-1275-5_8

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-010-7066-9

  • Online ISBN: 978-94-009-1275-5

  • eBook Packages: Springer Book Archive

Publish with us

Policies and ethics