Abstract
Although it is still not possible to integrate completely the various components involved in the inflammatory response, we now have the capability of indicating some of the effector pathways and identifying a number of points of interaction between them. The effector pathway considered in this chapter is the complement system, an essential part of the normal immune response. It consists of a special group of normal serum proteins which on activation interact sequentially in a process of limited proteolysis with the development of active macromolecules and fragments. Appreciation of this complex system has already provided a better understanding of certain pathological processes, the introduction of new therapeutic approaches, and the development of procedures to evaluate disease activity.
This work was supported by NIH Grants No. CA16247 and AI18309.
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References
Abell, E., and Black, M. M., 1974, Immunoglobulin and complement deposits in the skin in inflammatory facial dermatoses—an immunofluorescence study, Br. J. Dermatol. 91 (3): 281.
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.
Agnello, V., Koffler, D., Eisenberg, J. W., Winchester, R. J., and Kunkel, H. G., 1971, Clq precipitins in the sera of patients with systemic lupus erythematosus and other hypocomplementemic states: Characterization of high and low molecular weight types, J. Exp. Med. 134: 228.
Agnello, V., de Bracco, M. M., and Kunkel, H. G., 1972, Hereditary C2 deficiency with some manifestations of systemic lupus erythematosus, J. Immunol. 108: 837.
Alarcon-Segovia, D., and Ramos-Niembro, F., 1976, Association of partial lipodystrophy and Sjogren’s syndrome, Ann. Int. Med. 85: 474.
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 (7): 349.
Alper, C. A., Cotten, H. R., Rosen, F. S., Rabson, A. R., Macnab, G. M., and Gear, J. S., 1972a, Homozygous deficiency of C3 in a patient with repeated infections, Lancet 2: 1179.
Alper, C. A., Rosen, F. S., and Lachman, P. J., 19726, Inactivation of the third component of complement by an inhibitor in the properdin pathway, Proc. Natl. Acad. Sci. USA 69: 2910.
Alper, C. A., Goodkofsky, I., and Lepow, I. H., 1973a, The relationship of glycine-rich g glycoprotein to factor B in the properdin system and to cobra factor-binding protein of human serum, J. Exp. Med. 137: 424.
Alper, C. A., Bloch, K. J., and Rosen, F. S., 1973b, Increased susceptibility to infection in a patient with type II essential hypercatabolism of C3, N. Engl. J. Med. 288: 601.
Alpert, E., Isselbacher, K. J., and Schur, P. H., 1971, The pathogenesis of arthritis associated with viral hepatitis: Complement-component studies, N. Engl. J. Med. 285: 185.
Arroyave, C. M., and Müller-Eberhard, H. J., 1973, Interactions between human C5, C6 and C7 and their functional significance in complement-dependent cytolysis, J. Immunol. 111: 536.
Arroyave, C. M., and Tan, E. M., 1976, Detection of complement activation by counter immunoelectrophoresis, J. Immunol. Methods 13: 101.
Augener, W., Grey, H. M., Cooper, N. R., and Müller-Eberhard, H. J., 1971, The reaction of monomeric and aggregated immunoglobulins with CI, Immunochemistry 8: 1111.
Baart De la Faille, H., 1975, Erythropoietic protoporphyria: A photodermatosis, thesis defense, Oosthoek, Schelthema and Holkema, Utrecht.
Ballow, M., Ward, G. W., Gershwin, M. E., and Day, N. K., 1975a, C1 bypass complement activation in patients with chronic urticaria and angioedema, Lancet 2: 248.
Ballow, M., Ward, G. M., Wilbur, R., and Day, N. K., 1975b, Abnormal serum complement profile in patients with idiopatic urticaria, J. Allergy Clin. Immunol. 55: 105.
Ballow, M., Shira, J. E., Harden, L., Yang, S. Y., and Day, N. K., 1975c, Complete absence of the third component of complement in man, J. Clin. Invest. 56: 703.
Bennett, W. M., Bardana, E. J., Wuepper, K., Houghton, D., Border, W. A., Götze, D., and Schreiber, R., 1977, Partial lipodystrophy, C3 nephritic factor and clinically inapparent mesangiocapillary glomerulonephritis, Am. J. Med. 62: 757.
Berrens, L., Jankowski, E., and Jankowski-Berntsen, I., 1976, Complement component profiles in urticaria, dermatitis herpetiformis, and alopecia areata, Br. J. Dermatol. 95 (2): 145.
Beutner, E. H., Cherzolski, T. P., Bean, S. F., and Jordan, R. E., (eds.), 1973, Immunopathology of the Skin: Labeled Antibody Studies, Dowden, Hutchinson and Ross, Stroudsburg, Pa.
Beutner, E. H., Jarzabek-Chorzelska, M., Jablonska, S., and Chorzelski, T. P., 1978, Autoimmunity in psoriasis: A complement immunofluorescence study, Arch. Dermatol. Res. 261 (2): 123.
Bianco, C., Patrick, R., and Nussenzweig, V., 1970„ Population of lymphocytes bearing a membrane receptor for antigen antibody complexes. 1. Separation and characterization, J. Exp. Med. 132: 702.
Bianco, C., Dukor, P., and Nussenzweig, V., 1971, Follicular localization of antigen: Possible role of lymphocytes bearing a receptor for antigen-antibody complement complexes, in: Morphological and Functional Aspects of Immunity ( K. Lindahl-Kiessling, G. Alm, and G. M. Hanna, Jr., eds.), p. 251, Plenum Press, New York.
Bladen, H. A., Gewurz, H., and Mergenhaggen, S. E., 1997, Interaction of the complement system with the surface and endotoxic lipopolysaccharide of Veillonella alcalescens, J. Exp. Med. 125: 767.
Bokisch, V. A., and Muller-Eberhard, H. J., 1970, Anaphylatoxin inactivator of human plasma: Its isolation and characterization as a carboxypeptidase, J. Clin. Invest. 49: 2427.
Bokisch, V. A., Cochrane, C. G., and Müller-Eberhard, H. J., 1969, Isolation of a fragment (C3a) of the third component of human complement containing anaphylatoxin and chemotactic activity and description of an anaphylatoxin inactivator of human serum, J. Exp. Med. 129: 1109.
Bokisch, V. A., Top, F. H., Russell, P. K., Dixon, F. J., and Müller-Eberhard, H. J., 1973, The potential role of complement in dengue hemorrhagic shock syndrome, N. Engl. J. Med. 289: 996.
Bofotin, C., Morris, S., Tack, B., and Prahl, J., 1977, Purification and structural analysis of the fourth component of human complement, Biochemistry 16: 2008.
Borsos, T., Rapp, H. J., and Mayer, M. M., 1961a, Studies on the second component of complement. I. The reaction between EACI, 4 and C2: Evidence on the single site mechanism of immune hemolysis and determination of C2 on a molecular basis, J. Immunol. 87: 310.
Borsos, T., Rapp, H. J., and Mayer, M. M., 1961b, Studies on the second component of complement. II. The nature of the decay of EAC 142, J. Immunol. 87: 326.
Borsos, T., Dormashkin, R. R., and Humphrey, J. H., 1964, Lesions in erythrocytic membranes caused by immune haemolysis, Nature (London) 4929: 251.
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.
Braun-Falco, O., Maciejewski, W., Schmoeckel, C., and Scherer, R., 1977a, Inynunoelectronmicroscopical demonstration of in vivo bound complement C3 in psoriatic lesions, Arch. Dermatol. Res. 260: 57.
Braun-Falco, O., Mannel, C., and Scherer, R., 1977b, Demonstration of circulating soluble immune complexes in the sera of psoriatic patients using the 1251-CI Q deviation test, Hautarzt 28: 658.
Breverman, I. M., 1970, The Angiitides: Skin Signs of Systemic Diseases, p. 199, Saunders, Philadelphia.
Calcott, M. A., and Müller-Eberhard, H. J., 1972, Clq protein of human complement, Biochemistry 11: 3443.
Caldwell, R. J., Ruddy, S., Schur, P. H., and Austen, K. F., 1972, CI inhibitor deficiency in lymphosarcoma, Clin. Immunol. Immunopathol. 1: 39.
Chorzelski, T. P., Jablonska, S., Blaszczyk, M., and Trifthouser, C., 1971, Immunofluorescent studies in the diagnosis of dermatitis herpetiformis and its differentiation from bullous pemphoid, J. Invest. Dermatol. 56: 371.
Cochrane, C. G., and Müller-Eberhard, H. J., 1998, The derivation of two distinct anaphylatoxin activities from the third and fifth component of human complement, J. Exp. Med. 127: 371.
Cohen, S. H., Koethe, S. M., et al.,1978, Acquired angioedema associated with rectal carcinoma and its response to danazol therapy, J. Allergy Clin. Immunol. 62:217.
Conradie, J. D., Volauakis, J. E., and Stroud, R. M., 1975, Evidence for a serum inhibitor of Clq, Immunochemistry 12: 967.
Cooper, N. R., 1969, Immune adherence by the fourth component of complement, Science 165: 396.
Cooper, N. R., 1975a, Enzymatic activity of the second component of complement, Biochemistry 14: 4245.
Cooper, N. R., 1975b, Isolation and analysis of the mechanism of action of an inactivator of C4b in normal human serum, J. Exp. Med. 141: 890.
Cooper, N. R., tenBensel, 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 quantification of C2, J. Immunol. 101: 1176.
Costanzi, J. J., Coltman, C. A., and Donaldson, V. H., 1969, Activation of complement by a monoclonal cry-oglobulin associated with cold urticaria, J. Lab. Clin. Med. 74: 902.
Curd, J. G., Ziccardi, R. J., Kaplan, R., Suridsmo, J. S., Stevenson, D. D., Müller-Eberhard, H. J., and Vaughn, J. H., 1978, The syndrome of Clq deficiency, chronic urticaria, and vasculitis, presented at The Asthma and Allergic Diseases Centers Meeting, NIH, December, Bethesda, Md.
Daha, M. R., Fearon, D. T., and Austen, K. F., 1976, C3 nephritic factor (C3NeF): Stabilization of fluid phase and cell bound alternative pathway convertase, J. Immunol. 116: 1.
Dahl, M. G., and McGibbon, D. H., 1976, Complement in inflammatory acne vulgaris letter, Br. Med. J. 2: 1383.
Daniels, C. A., Borsos, T., Rapp, H. J., Snyderman, R., and Notkins, A. L., 1969, Neutralization of sensitized virus by the fourth component of complement, Science 165: 508.
Daniels, C. A., Borsos, T., Rapp, H. J., Snyderman, R., and Notkins, A. L., 1970, Neutralization of sensitized virus by purified components of complement, Proc. Natl. Acad. Sci. USA 65: 528.
Day, N. K., Geiger, H., Stroud, R., deBracco, M., Man-cado, B., Windhorst, D., and Good, R. A., 1972, Clr deficiency: An inborn error associated with cutaneous and renal disease, J. Clin. Invest. 51: 1102.
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.
Day, N. K., L’Esperance, P., and Siniscalco, M., 1975a, Linkage between hereditary C2 deficiency and HL-A, J. Exp. Med. 141: 1464.
Day, N. K., Winfield, J. B., Winchester, R. J., Gee, T. S., and Kunkel, H. G., 1975b, Evidence for immune-complexes involving anti-lymphocyte antibodies associated with hypocomplementemia in chronic lymphocytic leukemia, Clin. Res. 23: 410.
Dias Da Silva, W., Eisele, J. W., and Lepow, I. H., 1967, Complement as a mediator of inflammation. III. Purification of the activity with anaphylatoxin properties generated by interaction of the first four components of complement and its identification as a cleavage product of C’3, J. Exp. Med. 126: 1027.
Donaldson, V. H., and Evans, R. R., 1963, A biochemical abnormality in hereditary angioneurotic edema: Absence of serum inhibitor of C’1 esterase, Am. J. Med. 35: 37.
Donaldson, V. H., Rosen, F. S., and Bing, D. H., 1977, Role of the second component of complement and plasmin in kinin release in hereditary angioneurotic edema, Clin. Res. 25: 520A.
Dukor, P., and Hartmann, K. H., 1973, Bound C3 as the second signal for B-cell activation, Cell. Immunol. 7: 349.
Eden, A., Miller, G. W., and Nussenzweig, V., 1973, Human lymphocytes bear membrane receptors for C3b and C3d, J. Clin. Invest. 52: 3239.
Ehlenberger, A. G., and Nussenzweig, V., 1975, Synergy between receptors for FC and C3 in the induction of phagocytosis by human monocytes and neutrophils, Fed. Proc. 34: 854.
Eisenger, A. J., Shortland, J. R., and Moorhead, P. J., 1972, Renal disease in partial lipodystrophy, Q. J. Med. 41: 343.
Epstein, J. H., Tuffanelli, D. L., and Epstein, W. L., 1973, Cutaneous changes in the porphyrias—A microscopic study, Arch. Dermatol. 107: 684.
Fearon, D. T., 1978, Regulation by membrane sialic acid of ß1H-dependent decay-dissociation of amplification C3 convertase of the alternative complement pathway, Proc. Natl. Acad. Sci. USA 75: 1971.
Fearon, D. T., and Austen, K. F., 1975a, Properdin: Building to C3b and stabilization of the C3b dependent C3 convertase, J. Exp. Med. 142: 856.
Fearon, D. T., and Austen, K. F., 1975b, Properdin: Initiation of alternative complement pathway, Proc. Natl. Acad. Sci. USA 72: 3220.
Fearon, D. T., and Austen, K. F., 1977, Activation of the alternative complement pathway due to resistance of zymosan-bound amplification convertase to endogenous regulatory mechanisms, Proc. Natl. Acad. Sci. USA 74: 1683.
Fearon, D. T., Austen, K. F., and Ruddy, S., 1973, Formation of a hemolytically active cellular intermediate by the interaction between properdin factors B and D and the activated third component of complement, J. Exp. Med. 138: 1305.
Fearon, D. T., Daha, M. R., Weiler, J. M., and Austen, K. F., 1976, The natural modulation of the amplification phase of complement activation, Transplant. Rev. 32: 12.
Fearon, 1978, Activation of the alternative complement pathway by Escherichia coli: Resistance of bound C3b to inactivation by C3bINA and ß1H, J. Immunol. 120: 1772.
Feig, P. U., Soter, N. A., Yager, H. M., Caplan, L., and Rosen, S., 1976, Vasculitis with urticaria, hypocomplementemia, and multiple system involvement, J. Am. Med. Assoc. 236: 2065.
Fernandez, N. O., Henson, P. M., Otani, A., and Hugli, T. E., 1978, Chemotactic response to human C3a and C5a anaphylatoxins. 1. Evaluation of C3a and C5a leukotaxis in vitro and under simulated in vivo conditions, J. Immunol. 120: 109.
Friend, P., Repine, J. E., Kim, Y., Clawson, C. C., and Michael, A. F., 1975, Deficiency of the second component of complement (C2) with chronic vasculitis, Ann. Int. Med. 83: 813.
Fu, B. S. M., Kunkel, H. G., Brusman, H. P., Allen, F. H., Jr., and Fotino, M., 1974, Evidence for linkage between HL-A histocompatibility genes and those involved in the synthesis of the second component of complement, J. Exp. Med. 140: 1108.
Fujita, T., Gigli, I., and Nussenzweig, V., 1978, Human C4-binding protein. H. Role in proteolysis of C4b by C3b-inactivator, J. Exp. Med. 148: 1044.
Geha, R. S., and Alk, K. F., 1976, Skin lesions, angioedema, eosinophilia, and hypocomplementemia, J. Pediatr. 89: 724.
Gelfand, E. W., Clarkson, J. E., and Minta, J. O., 1975, Selective deficiency of the second component of complement in a patient with anaphylactoid purpura, Clin. Immunol. Immunopathol. 4: 269.
Gelfand, J. A., Sherins, R. J., Ailing, D. W., and Frank, M. M., 1976, Treatment of hereditary angioedema with Danazol: Reversal of clinical and biochemical abnormalities, N. Engl. J. Med. 295: 1444.
Gellis, S., Green, S., and Walker, D., 1958, Chronic renal disease in children with lipodystrophy, Am. J. Dis. Child. 96: 605.
Gewurz, A., and Lint, T. F., 1978, Homozygous C2 deficiency with fulminant lupus erythematosus: Severe nephritis via the alternative complement pathway, Arthritis Rheum. 21 (1): 28.
Gewurz, H., Pickering, R. J., Mergenhagen, S. E., and Good, R. A., 1966, The complement profile in acute glomerulonephritis, systemic lupus erythematosus, and hypocomplementemic glomerulonephritis, Int. Arch. Allergy Appl. Immunol. 34: 556.
Gewurz, H., Pickering, R. J., Snyderman, R., Lichtenstein, L. M., and Good, R. A., 1970, Interaction of the complement system with endotoxin lipopolysaccharide in immunoglobulin-deficient sera, J.. Exp. Med. 131: 817.
Ghebrehiwet, B., and Müller-Eberhard, H. J., 1979, C3e: An acidic fragment of human C3 with leukocytosis-inducing activity, J. Immunol. 123: 616.
Gigli, I., and Austen, K. F., 1969, Fluid phase destruction of C4 by Cl: Its enhancement and inhibition by homologous and heterologous C4, J. Exp. Med. 129: 679.
Gigli, I., and Nelson, R. A., 1968, Complement dependent immune phagocytosis. 1. Requirement for CI, C4, C2, and C3, Exp. Cell Res. 51: 45.
Gigli, I., Ruddy, S., and Austen, K. F., 1968, The stoichiometric measurement of the serum inhibitor of the first component of complement by inhibition of immune hemolysis, J. Immunol. 100: 1154.
Gigli, I., Mason, J. W., Colman, R. W., and Austen, K. F., 1970, Interaction of plasma kallikrein with the CT inhibitor, J. Immunol. 104: 574.
Gigli, I., Schothorst, A., Soter, N. A., and Pathek, M., 1974, Photoinhibition of the complement system in erythropoietic protoporphyria, Clin. Res. 22: 418a.
Gigli, I., Koethe, S., and Austen, K. F., 1975, Participation of an early component of complement and Hage-man factor in C3 destruction by zymosan, Clin. Immunol. Immunopathol. 4: 189.
Gigli, I., Porter, R., and Sim, R. B., 1976, The unactivated form of the first component of human complement, Cl, Biochem. J. 157: 541.
Gigli, I., Von Zabern, I., and Porter, R. R., 1977, The isolation and structure of C4, the fourth component of human complement, Biochem. J. 165: 439.
Gigli, I., Fujita, T., and Nussenzweig, V., 1979, Modulation of the classical pathway C3 convertase by the plasma proteins C4-binding protein and C3b inactiva-tor, Proc. Natl. Acad. Sci. USA 76: 6596.
Giraldo, G., DeGos, L., Beth, E., Sasportes, M., Mar-celli, A., Harbi, R., and Day, N. K., 1977, C8 deficiency in a family with xeroderma pigmentosum. Lack of linkage to the HLA region, Clin. Immunol. Immunopathol. 8: 377.
Goldman, J. N., Ruddy, S., and Austen, K. F., 1972, Reaction mechanisms of nascent C567 (reactive lysis). I. Reaction characteristics for production of EC567 and lysis by C8 and C9, J. Immunol. 109: 353.
Goldstein, I. M., and Gigli, I., 1980, Recurrent meningococcal meningitis in a patient with complete absence of the sixth component of complement, ms. submitted for publication.
Goldstein, I. M., Kaplan, H. B., Radin, A., and Frosch, M., 1976, Independent effects of IgG and complement upon human polymorphonuclear leukocyte function, J. Immunol. 117: 1282.
Götze, O., and Müller-Eberhard, H. J., 1976, The alternative pathway of complement activation, Adv. Immmunol. 24: 1.
Gower, R., Sausker, W., Kohler, P., Thorne, G. G., and McIntosh, R., 1978, Small vessel vasculitis caused by hepatitis B virus immune complexes, J. Allergy Clin. Immunol. 62: 222.
Granerus, G., Hallberg, L., Laurell, A. B., and Wetterguist, H., 1967, Studies on the histamine metabolism and the complement system in hereditary angioneurotic edema, Acta Med. Scand. 182: 11.
Hauptmann, G., Groshans, E., and Heid, E., 1974, Lupus erythematoux aigus et deficits hereditaires en complement. A propos d’un cas par deficit complet en C4, Ann. Dermatol. 101: 479.
Huber, H., Polley, M. J., Linscott, W. D., Fudenberg, H. H., and Müller-Eberhard, H. J., 1968, Human monocytes: Distinct receptor sites for the third component of complement and for immunoglobulin G, Science 162: 1281.
Hunsicker, L. G., Ruddy, S., Carpenter, C. B., Schur, P. H., Merrill, J. P., Müller-Eberhard, H. J., and Austen, F., 1972, Metabolism of the third complement (C3) in nephritis, N. Engl. J. Med. 287: 835.
Hunsicker, L. G., Ruddy, S., and Austen, K. F., 1973, Alternate complement pathway: Factors involved in cobra venom factor (CVF) activation of the third component of complement, J. Immunol. 110: 128.
Ishizaka, T., Ishizaka, K., Borsos, T., and Rapp, H., 1966, Cl fixation by human isoagglutinin: Fixation of CI by YG and YM but not YA antibody, J. Immunol. 97: 716.
Jablonska, S., Chorzelski, P., Jarzabek-Chorzelska, M., and Beutner, E. H., 1976, Immunofluorescent findings in psoriasis of the scalp, in: Biology and Disease of the Hair ( K. Toda, ed.), p. 309, University Park Press, Baltimore.
Jablonska, S., Chorzelski, T. P., Beutner, E. H., Maciejowska, E., Abek-Chorzelska, X., and Esa, G., 1978, Autoimmunity in psoriasis. Relation of disease activity and forms of psoriasis to immunofluorescence findings, Arch. Dermatol. Res. 261: 135.
Jamison, T. H., Cooper, N. M., and Epstein, W. V., 1978, Lichen planus and discoid lupus erythematosus: Overlap syndrome associated with cryoglobulinemia and hypocomplementemia, Arch. Dermatol. 114: 1039.
Jasin, H. E., 1977, Absence of the eighth component of complement in association with systemic lupus erythematosus-like disease, J. Clin. Invest. 60: 709.
Jensen, J., 1967, Anaphylatoxin in its relation to the complement system, Science 155: 1122.
Jordan, R. E., and Provost, T. T., 1978, Bullous diseases of the skin, in: Immunological Diseases, Vol. 2 (M. Sauster, ed.), Little, Brown, New York.
Jordan, R. E., Day, N. K., Sams, W. M., and Good, R. A., 1973a, The complement system in bullous pemphigoid. I. Complement and component level in sera and blister fluid, J. Clin. Invest. 52: 1207.
Jordan, R. E., Day, N. K., Luckasen, J. R., and Good, R. A., 1973b, Complement activation in pemphigus vulgaris blister fluid, Clin. Exp. Immunol. 15: 53.
Jordan, R. E., McDuffe, F. C., Good, R. A., and Day, N. K., 1974, Diffuse normolipaemic plane xanthomatosis, Clin. Exp. Immunol. 18: 407.
Kagan, L. J., 1964, Some biochemical and physical properties of the human permeability globulins, Br. J. Exp. Pathol. 45: 604.
Kerr, M. A., and Porter, R. R., 1978, The purification and properties of the second component of human complement, Biochem. J. 171: 99.
Kikindjanin, V., and Milakov, J., 1976, Serum immunoglobulin and complement levels in patients with psoriasis vulgaris, Allerg. Immunol. 22 (2): 143.
Kim, Y., Friend, P. S., Dresner, I. G., Yunis, E. J., and Michael, A. F., 1977, Inherited deficiency of the second component of complement (C2) with membranoproliferative glomerulonephritis, Am. J. Med. 62: 765.
Klemperer, M. R., Woodworth, H. C., Rosen, F. S., and Austen, K. F., 1966, Hereditary deficiency of the second component of complement (C2) in man, J. Clin. Invest. 45: 880.
Kohler, P. F., 1973, Clinical immune complex disease: Manifestations in systemic lupus erythematosus and hepatitis B virus infection, Medicine (Baltimore) 52: 419.
Kohler, P. F., Percy, J., Wookrow, M. C., and Smyth, C. J., 1974, Hereditary angioedema and familiar lupus erythematosus in identical twin boys, Am. J. Med. 56: 406.
Kolb, W. P., Haxby, J. A., Arroyave, C. M., and Muller-Eberhard, H. J., 1972, Molecular analysis of the membrane attack mechanism of complement, J. Exp. Med. 135: 549.
Lachmann, P. J., and Müller-Eberhard, H. J., 1968, The demonstration in human serum of conglutinogen-activity factor and its effect on the third component of complement, J. Immunol. 100: 691.
Lachmann, P. J., and Thompson, R. A., 1970, Reactive lysig: The complement-mediated lysis of unsensitized cells. II. The characterization of activated reactor as C5b and the participation of C8 and C9, J. Exp. Med. 131: 643.
Lachmann, P. J., Hobart, M. J., and Woo, P., 1978, Combined genetic deficiency of C6 and C7 in man, Clin. Exp. Immunol. 33: 193.
Laurell, C. B., 1966, Quantitative estimation of proteins by electrophoresis in agarose gel containing antibodies, Anal. Biochem. 15: 45.
Laurell, C. B., Johnson, U., Martensson, U., and Sjohlm, A. G., 1978, Formation of complexes composed of CT, CTs s and CTinactivator inactivator in human serum on activation of Cl, Acta Pathol. Microbiol. Scand. 86C: 299.
Lay, W. H., and Nussenzweig, V., 1968, Receptors for complement on leukocytes, J. Exp. Med. 128:991.
Lazarus, G. S., Yost, F. J., and Thomas, C. A., 1977, Polymorphonuclear leukocytes: Possible mechanism of accumulation in psoriasis, Science 198: 1162.
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.
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.
Lehner, T., 1968, Autoimmunity in oral diseases, with special reference to recurrent oral ulceration, Proc. R. Soc. Med. 61: 515.
Lepow, I. H., Ratnoff, O. J., and Pillemer, L., 1956, Elution of an esterase from antigen-antibody aggregates treated with human complement, Proc. Soc. Exp. Biol. Med. 92: 32.
Lepow, I. H., Noff, G. B., Todd, E., Pensky, J., and Hinz, C. F., Jr., 1963, Chromatographic resolution of the first component of human complement into three activities, J. Exp. Med. 117: 983.
Lepow, I. H., Dias da Silva, W., and Eisele, J. W., 1968, Nature and biological properties of human anaphylatoxin, in: Biochemistry of the Acute Allergic Reactions ( K. F. Austen and E. L. Becker, eds.), Davis, Philadelphia.
Lepow, I. H., Dias da Silva, W., and Patrick, R. A., 1969, Biologically active cleavage products of components of complement, in: Cellular and Humoral Mechanisms in Anaphylaxis and Allergy ( H. Z. Movat, ed.), p. 237, Karger, Basel.
Lepow, I. H., Willms-Kretschmer, K., and Patrick, R. A., 1970, Gross and ultrastructural observations on lesions produced by intradermal injection of human C3a in man, Am. J. Pathol. 61: 13.
Levinsky, R. J., and Lehner, T., 1978, Immune complexes in oral ulcerations and Behçet’s syndrome, Clin. Exp. Immunol. 32: 190.
Levy, L. R., and Lepow, I. H., 1959, Assay and properties of serum inhibitor of C1-esterase, Proc. Soc. Exp. Biol. Med. 101: 608.
Lim, D., Gewurz, A., Ghaze, M., Lint, T. F., Sephari, B., and Gewurz, H., 1976, Absence of the sixth component of complement in a patient with repeated episodes of meningococcal meningitis, J. Pediatr. 89: 42.
Lint, T. F., Behrends, C. L., and Gewurz, H., 1977, Serum lipoproteins and C567-INH activity, J. Immunol. 119: 883.
Louie, J. S., and Glovsky, M., 1975, Complement determinations in the synovial fluid and serum of a patient with erythema nodosum leprosum, Int. J. Lepr. 43 (3): 252.
Mahler, F., Intaglietta, M., Hugli, T. E., and Johnson, A. R., 1975, Influences of C3a anaphylatoxin compared to other vasoactive agents on the microcirculation of the rabbit omentum, Microvasc. Res. 9: 345.
Mancini, G., Carbonara, A. O., and Heremans, J. F., 1%5, Immunochemical quantitation of antigens by single radial immunodiffusion, Immunochemistry 2: 235.
Marcus, R. L., Shin, H. S., and Mayer, M. M., 1971, An alternate complement pathway: C3 cleaving activity, not due to C4,C2a, on endotoxin lipopolysaccharide after treatment with guinea pig serum: Relation to proper-din complement components, Proc. Natl. Acad. Sci. USA 68: 1351.
Marder, R. J., Rent, R., Choi, E. J., and Gewurz, H., 1976, Selective Clq deficiency associated with urticaria-like lesions and cutaneous vasculitis, Am. J. Med. 61: 560.
Marshall, D. A., Nesbitt, L. T., and Biundo, J. J., 1974, Serum complement related to skin lesions of systemic lupus erythematosus, South. Med. J. 67: 1275.
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.
Mayer, M. M., 1964, Complement and complement fixation, in: Experimental Immunochemistry ( E. A. Kabat and M. M. Mayer, eds.), p. 133, Thomas, Springfield, Ill.
Mayer, M. M., 1972, Mechanism of cytolysis by complement, Proc. Natl. Acad. Sci. USA 69: 2954.
McConnell, I., Klein, G., Lint, T. F., and Lachmann, P. J., 1978, Activation of alternative complement pathway by human B-cell lymphoma lines is associated with Epstein-Barr transformation of cells, Eur. J. Immunol. 8: 453.
McDuffie, F. C., Sams, W. M., Jr., Maldonado, J. E., Andreini, P. H., Conn, D. L., and Samayoa, E. A., 1973, Hypocomplementemia with cutaneous vasculitis and arthritis. Possible immune complex syndrome, Mayo Clin. Proc. 48: 340.
McLeod, B., Baker, P., and Gewurz, H., 1975, Studies on the inhibition of C56 initiated lysis (reactive lysis). III. Characterization of the inhibitor activity C567-INH and its mode of action, Immunology 28: 133.
Medicus, R. G., Schreiber, R. D., Götze, O., and Müller-Eberhard, H. J., 1976, A molecular concept of the properdin pathway, Proc. Natl. Acad. Sci. USA 73: 612.
Miescher, P. A., Paronetto, F., and Kollier, D., 1965, Immunofluorescent studies in human vasculitis, in: Immunopathology IV International Symposium ( P. Grabar and P. Miescher, eds.), p. 446, Grune and Stratton, New York.
Milgrom, R., Curd, J. G., Stevenson, D. D., Mathison, D. A., and Vaughn, J. H., 1978, Iodide sensitivity in patients with chronic idiopathic urticaria or systemic lupus erythematosus, presented at the Asthma and Allergic Diseases Center, December, NIH, Bethesda, Md.
Miller, G. W., and Nussenzweig, V., 1974, Complement as regulator of interactions between immune-complexes and cell membranes, J. Immunol. 113: 464.
Miller, M. E., and Nilsson, U. R., 1970, A familial deficiency of the phagocytosis-enhancing activity of serum related to a dysfunction of the fifth component of complement (C5), N. Engl. J. Med. 282 (7): 354.
Miller, M. E., and Nilsson, U. R., 1974, A major role of the fifth component of complement in the opsonization of yeast particles. Partial dichotomy of function and immunochemical measurement, Clin. Immunol. Immunopathol. 2: 246.
Mills, B. J., and Cooper, N. R., 1978, Antibody-independent neutralization of vesicular stomatitis virus by human complement, J. Immunol. 121: 1549.
Minta, J. O., and Lepow, I. H., 1974, Physical and chemical studies of human properdin purified by elution from zymosan and affinity chromatography, Immunochemistry 11: 361.
Moncada, B., Day, N. K. B., Good, R. A., and Windhorst, D. B., 1972, Lupus-erythematosus-like syndrome with a familial defect of complement, N. Engl. J. Med. 286: 689.
Montovani, B., 1975, Different roles of IgG and complement receptors in phagocytosis by polymorphonuclear leukocytes, J. Immunol. 115: 15.
Montovani, B., Rabinovitch, M., and Nussenzweig, V., 1972, Phagocytosis of immune complexes by macrophages. Different roles of the macrophage receptor sites from complement (C3) and for immunoglobulin (IgG), J. Exp. Med. 135: 780.
Müller-Eberhard, H. J., 1975, Complement, Annu. Rev. Biochem. 44: 697.
Müller-Eberhard, H. J., and Biro, E., 1967, Isolation and description of the fourth component of human complement, J. Exp. Med. 118: 447.
Müller-Eberhard, H. J., and Goetz, O., 1972, C3 proactivator convertase and its mode of action, J. Exp. Med. 135: 1003.
Müller-Eberhard, H. J., and Lepow, I. H., 1965, Cl esterase effect on activity and physicochemical properties of the fourth component of complement, J. Exp. Med. 121: 819.
Müller-Eberhard, H. J., Dalmasso, A. P., and Calcott, M. A., 1966, The reaction mechanism of 1.3 IC globulin in immune hemolysis, J. Exp. Med. 123: 33.
Müller-Eberhard, H. J., Polley, M. H., and Calcott, M. A., 1967, Formation and functional significance of a molecular complex derived from the second and fourth components of human complement, J. Exp. Med. 125: 359.
Naff, G. B., and Ratnoff, O. D., 1968, The enzymatic nature of CIr. Conversion of CI to Cl esterase and digestion of amino acid esters by Clr, J. Exp. Med. 128: 571.
Naff, G. B., Pensky, J., and Lepow, I. H., 1964, The macromolecular nature of the first component of human complement, J. Exp. Med. 119: 593.
Nagasawa, S., and Stroud, R. M., 1977, Cleavage of C2 by C 1 s into the antigenically distinct fragments C2a and C2, Proc. Natl. Acad. Sci. USA 74: 2998.
Nelson, R. A., 1953, The immune adherence phenomenon: An immunologically specific reaction between microorganisms and erythrocytes leading to the enhancement of phagocytosis, Science 118: 733.
Newman, S. L., Vogler, L. B., Feigin, R. D., and Johnston, R. B., Jr., 1978, Recurrent septicemia associated with congenital deficiency of C2 and partial deficiency of factor B and the alternative complement pathway, N. Engl. J. Med. 299 (6): 290.
Nilsson, U. R., and Mapes, J., 1973, Polyacrylamide gel electrophoresis (PAGE) of reduced and dissociated C3 and C5: Studies of the polypeptide chains (PPC) subunits and their modifications by trypsin and C42–C423, J. Immunol. 111: 293.
Oberling, F., Hauptmann, G., Lang, J. M., North, M. L., Mayer, G., and Lachman, P., 1976, Acquired C-1-inhibitor deficiency in lymphoproliferative disease with serum immunoglobulin abnormality, Blut 32: 195.
Ochs, H. D., Rosenfeld, S. I., Thomas, D. E., Giblett, E., Alper, C. A., Dupont, B., Schaller, J. G., Gilliland, B. C., Hansen, J. A., and Wedgwood, R. J., 1977, Linkage between the gene (or genes) controlling synthesis of the fourth component of complement and the major histocompatibility complex, N. Engl. J. Med. 296: 470.
Ochs, H. D., Jackson, C. G., Frank, M. M., Hosea, S. W., and Wedgwood, R. J., 1978, Antibody responses to a T cell dependent antigen in C4 deficiency, J. Immunol. 120: 1789.
Oishi, M., Takano, M., Miyachi, K., Ichikawa, Y., and Homma, M., 1976, A case of unusual SLE-related syndrome characterized by erythema multiforme, angioneurotic edema and marked hypocomplementemia, Int. Arch. Allergy Appl. Immunol. 50: 463.
Onion, D. K., Crumpacker, C. S., and Gilliland, B. C., 1971, Arthritis of hepatitis associated with Australia antigen, Ann. Intern. Med. 75: 29.
Osier, A. G., Randall, H. G., Hill, B. M., and Ovary, Z., 1959a, Some relationship between complement, passive cutaneous anaphylaxis and anaphylatoxin, in: The Mechanics of Hypersensitivity ( J. H. Shaffer, ed.), p. 281, Little, Brown, Boston.
Oster, A. G., Randall, H. G., Hill, B. M., and Ovary, Z., 1959b, Studies on the mechanism of hypersensitivity phenomena. III. The participation of complement in the formation of anaphylatoxin, J. Exp. Med. 110: 311.
Osier, A. G., Oliveria, B., Shin, H. S., and Sandberg, A. L., 1969, The fixation of guinea pig complement by Y 1, Y2 immunoglobulins, J. Immunol. 102: 269.
Osier, W., 1888, Hereditary angio-neurotic edema, Am. J. Med. Sci. 95: 362.
Osofsky, S. G., Thompson, B. H., Lint, T. F., and Gewurz, H., 1977, Hereditary deficiency of the third component of complement in a child with fever, skin rash, and arthralgias: Response to transfusion of whole blood, J. Pediatr. 90: 180.
Osterland, C. K., Espinoza, L., Parker, L. P., and Schur, P. H., 1975, Inherited C2 deficiency and systemic lupus erythematosus: Studies on a family, Ann. Int. Med. 82: 323.
Pangburn, M. K., and Müller-Eberhard, H. J., 1978, Complement C3 convertase: Cell surface restriction of 131H control and generation of restriction on neuraminidase-treated cells, Proc. Natl. Acad, Sci. USA 75: 2416.
Pangburn, M. K., Schreiber, R. D., and Müller-Eberhard, H. J., 1977, Human complement C3b inactivator: Isolation, characterization, and demonstration of an absolute requirement for the serum protein ß 1H for cleavage of C3b and C4b in solution, J. Exp. Med. 146: 257.
Pensky, J., and Schwick, H. G., 1969, Human serum inhibitor of C’1 esterase: Identity, with a2-neuraminoglycoprotein, Science 163: 698.
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.
Pepys, M. B., 1974, Role of complement in induction of antibody production in vivo. Effect of cobra factor and other C3-reactive agents on thymus dependent and thymus independent antibody response, J. Exp. Med. 140: 126.
Peters, D. K., Williams, D. G., Charlesworth, J. A., Boulton-Jones, J. M., Sissons, J. G. P., Evans, D. J., Kourilsky, O., and Morel-Maroger, L., 1973, Mesangiocapillary nephritis, partial lipodystrophy and hypocomplementaemia, Lancet 2: 535.
Petersen, B. H., Graham, J. A., and Brooks, G. F., 1976, Human deficiency of the eighth component of complement. The requirement of C8 for serum Neisseria gonorrhoeae bactericidal activity, J. Clin. Invest. 57: 283.
Pfaffenbach, G., Lamm, M., and Gigli, I., 1978, Activation of complement by mouse IgA immune-complexes, Fed. Proc. 37: 1377.
Pickering, R. J., Naff, G. B., Stroud, R. M., Good, R. A., and Gewurz, H., 1970, Deficiency of C1r in human serum: Effects on the structure and function of macro-molecular C 1, J. Exp. Med. 131: 803.
Pickering, R. J., Michael, A. F., Jr., Herdman, R. C., Good, R. A., and Gewurz, H., 1971, The complement system in chronic glomerulonephritis: Three newly associated aberrations, J. Pediatr. 78: 30–43.
Pillemer, L., and Ecker, E. E., 1941, Anticomplementary factors in yeasts, J. Biol. Chem. 137: 139.
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 phenomena, Science 120: 279.
Pincus, S., Bianco, C., and Nussenzweig, V., 1972, Increased proportion of complement receptor lymphocytes in the peripheral blood of patients with chronic lymphatic leukemia, Blood 40: 303.
Platts-Mills, T. A. E., and Ishizaka, K., 1974, Activation of the alternate pathway of human complement by rabbit cells, J. Immunol. 113: 348.
Podack, E. R., Kolb, W. P., and Müller-Eberhard, H. J., 1978, The C5b-6 complex: Formation, isolation and inhibition of its activity by lipoprotein and the S protein of human serum, J. Immunol. 120: 1841.
Polley, M. J., and Müller-Eberhard, H. J., 1968, The second component of human complement: Its isolation, fragmentation by Cl-esterase and incorporation into C3 convertase, J. Exp. Med. 128: 533.
Pondman, K. W., Stoop, J. W., Cormane, R. H., and Hannema, A. J., 1968, Abnormal C1 in a patient with systemic lupus erythematosus, J. Immunol. 101: 811.
Poskitt, T.R., Fortwengler, H.P., Jr., and Lunskis, B.J., 1973, Activation of the alternate complement pathway by autologous red cell stroma, J. Exp. Med. 138: 715.
Provost, T. T., and Tomasi, T. B., Jr., 1974, Evidence for the activation of complement via the alternate pathway in skin diseases. H. Dermatitis herpetiformis, Clin. Immunol. Immunopathol. 3: 178.
Ratnoff, O. D., and Naff, G. B., 1967, The conversion of C’ 1 s to C’ 1 esterase by plasmin and trypsin, J. Exp. Med. 125: 337.
Ratnoff, O. D., Pensky, J., Ogston, D., and Naff, G. B., 1969, The inhibition of plasmin, plasma kallikrein, plasma permeability factor, and the Clr of complement by serum Cl esterase inhibitor, J. Exp. Med. 129: 315.
Reid, K. B. M., 1971, Complement fixation by the F(ab’)2 fragment of pepsin treated antibody, Immunology 20: 649.
Reid, K. B. M., 1974, A collagen-like amino acid sequence in a polypeptide chain of human Clq (a subcomponent of the first component of complement), Biochem. J. 141: 189.
Reid, K. B. M., and Porter, R. R., 1975, The structure and mechanism of activation of the first component of complement, Contemp. Top. Mol. Immunol. 4: 1.
Reid, K. B. M., Lowe, D. M., and Porter, R. R., 1972, Isolation and characterization of Clq, a subcomponent of the first component of complement, from human and rabbit sera, Biochem. J. 130: 749.
Rosen, F. S., Charache, P., Pensky, J., and Donaldson, V., 1965, Hereditary angioneurotic edema. Two genetic variants, Science 148: 957.
Rosenfeld, S. I., Stapls, P., and Leddy, J., 1975, Angioedema and hypocomplementemia. Unusual feature of lymphoma, J. Allergy Clin. Immunol. 55: 104.
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.
Ross, G. D., and Polley, M. J., 1975, Specificity of human lymphocyte complement receptors, J. Exp. Med. 141: 1163.
Rother, K., 1972, Leukocyte mobilizing factor: A new biological activity derived from the third component of complement, Eur. J. Immunol. 2: 550.
Ruddy, S., and Austen, K. F., 1969, C3 inactivator of man. I. Hemolytic measurement by the inactivation of cell bound C3, J. Immunol. 102: 533.
Ruddy, S., and Austen, K. F., 1971, C3b inactivator in man. II. Fragments produced by C3b inactivator cleavage of cell-bound or fluid phase C3b, J. Immunol. 107: 742.
Ruddy, S., Gigli, I., Sheffer, A. L., and Austen, K. F., 1968, The laboratory diagnosis of hereditary angioedema, in: Proceedings of the 6th International Congress of Allergology, Montreal 1%7 (A. Knight et al.,eds.), Excerpta Medica International Congress Series No. 162, Amsterdam.
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: Correlation of C2 haemolytic activity with immunochemical measurements of C2 protein, Immunology 18: 943.
Russell, P. K., Intavivat, A., and Kanchanipilant, S., 1969, Anti-dengue immunoglobulins and serum ß1c/a globulin levels in dengue shock syndrome, J. Immunol. 102: 412.
Rynes, R. I., Urizar, R. E., and Pickering, R. J., 1977, Genetic deficiency of the second component of complement (C2) associated with systemic lupus erythematosus, Am. J. Med. 63: 279.
Rynes, R. I., Urizar, R. E., and Pickering, R. J., 1978, Heterogeneity of the clinical syndrome in patients with systemic lupus erythematosus and genetic deficiency of the second component of complement, Clin. Exp. Immunol. 32: 59.
Saitz, E. W., Dierks, R. E., and Shepard, C. C., 1968, Complement and the second component of complement in leprosy, Int. J. Lepr. 36: 400.
Sakai, K., and Stroud, R. M., 1973, Purification, molecular properties, and activation of Cl proesterase, Clsl, J. Immunol. 110: 1010.
Sams, W. M., Jr., 1974, Hypocomplementemia with cutaneous vasculitis and arthritis: A new syndrome, Arch. Dermatol. 110: 290.
Sams, W. M., Jr., Claman, H. N., Kohler, P. F., McIntosh, R. W., Small, P., and Mass, M. F., 1975, Human necrotizing vasculitis: Immunoglobulins and complement in vessel walls of cutaneous lesions and normal skin, J. Invest. Dermatol. 64: 441.
Sandberg, A. L., Götze, O., Müller-Eberhard, H. J., and Oster, A. G., 1971, Complement utilization by guinea pig Yl and Y2 immunoglobulins through the C3 activator system, J. Immunol. 107: 920.
Sandberg, A. L., Wohl, S. M., and Mergenhagen, S. E., 1975, Lymphokine production by C36-stimulated B cells, J. Immunol. 115: 139.
Scharfstein, J., Ferreira, A., Gigli, I., and Nussenzweig, V., 1978, Human C4-binding protein. I. Isolation and characterization, J. Exp. Med. 148: 207.
Scherer, R., and Braun-Falco, O., 1976, Alternative pathway complement activation: A possible mechanism inducing skin lesions in benign gonococcal sepsis, Br. J. Dermatol. 95: 303.
Schorlemmer, H. U., and Allison, A. C., 1976, Effects of activated complement components on enzyme secretion by macrophages, Immunology 31: 781.
Schrager, M. A., and Rothfield, N. F., 1977, Pathways of complement activation in chronic discoid lupus: Serologic and immunofluorescence studies, Arthritis Rheum. 20: 637.
Schreiber, A. D., Kaplan, A. P., and Austen, K. F., 1973, Inhibition by Cl INH of Hageman factor fragment activation of coagulation, fibrinolysis, and kinin-generation, J. Clin. Invest. 52: 1402.
Schreiber, A. D., Sweiman, B., Atkins, P., Goldwein, F., Pietra, G., Atkinson, B., and Abdow, N. I., 1976, Acquired angioedema with lymphoproliferative disorder: Association of CT inhibitor deficiency with cellular abnormality, Blood 48: 567.
Schreiber, R. D., and Müller-Eberhard, H. J., 1974, Fourth component of human complement. Description of a three polypeptide chain structure, J. Exp. Med. 140: 1324.
Schreiber, R. D., Götze, O., and Müller-Eberhard, H. J., 1976a, Nephritic factor and initiating factor C3/C5 convertases, Fed. Proc. 35: 253 (abstr.).
Schreiber, R. D., Götze, O., and Müller-Eberhard, H. J., 19766, Nephritic factor: Its structure and function and its relationship to initiating factor of the alternative pathway, Scand. J. Immunol. 5: 705.
Schreiber, R. D., Pangburn, M. K., Lesavre, P. H., and Müller-Eberhard, H. J., 1978, Initiation of the alternative pathway of complement: Recognition of activators by bound C3b and assembly of the entire pathway from six isolated proteins, Proc. Natl. Acad. Sci. USA 75: 3948.
Schroeter, A. L., Copeman, P. W. M., Jordan, R. E., Sams, W. M., Jr., and Winkelmann, R. K., 1971, Immunofluorescence of cutaneous vasculitis associated with systemic disease, Arch. Dermatol. 104: 254.
Schur, P. H., and Becker, E. L., 1963, Pepsin digestion of rabbit and sheep antibodies. The effect on complement fixation, J. Exp. Med. 118: 891.
Seery, U. L., and Müller-Eberhard, H. J., 1973, Binding of porphyrins to rabbit hemopexin and albumin, J. Biol. Chem. 248: 3796.
Sequeira, M., and Nelson, R. A., 1961, Platelet agglutination by immune-complexes and its possible role in hypersensitivity, J. Immunol. 86: 516.
Sheagren, J. N., Block, J. B., Trautman, J. R., and Wolff, S. M., 1969, Immunologic reactivity in patients with leprosy, Ann. Intern. Med. 70: 295.
Sheffer, A. L., Craig, J. M., Willms-Kretschmer, K., Austen, K. F., and Rosen, F. S., 1971, Histopathological and ultrastructural observations on tissues from patients with hereditary angioneurotic edema, J. Allergy 47: 292.
Shelton, E., Yonemasa, K., and Stroud, R. M., 1972, Ultrastructure of the human complement component, Clq, Proc. Natl. Acad. Sci. USA 69: 65.
Shevach, E. M., Herberman, R., Frank, M. M., and Green, I., 1972, Receptors for complement and immunoglobulin on human leukemic cells and human lymphoblastoid cell lines, J. Clin. Invest. 51: 1933.
Shikano, S., 1966, Ocular pathology of Behçet’s syndrome, in: International Symposium on Behçet’s Disease, p. 111, Rome.
Shimada, K., Kogure, M., Kawashima, R., and Nishoka, K., 1974, Reduction of complement in Behçet’s disease and drug allergy, Med. Biol. 52: 234.
Shin, H. S., and Mayer, M. M., 1968, The third component of guinea pig complement. II. Kinetic study of the reaction of EAC 42a with guinea pig C3, Biochemistry 7: 2997.
Shin, H. S., Gewurz, H., and Snyderman, R., 1969, Reaction of cobra venom factor with guinea pig complement and generation of an activity chemotactic for polymorphonuclear leukocytes, Proc. Soc. Exp. Biol. Med. 131: 203.
Shiraishi, S., and Stroud, R. M., 1975, Cleavage products of C4b produced by enzymes in human serum, Immunochemistry 12: 935.
Sim, R. B., Porter, R. R., Reid, K. B. M., and Gigli, I., 1977, The structure and enzymatic activities of the Clr and C Is subcomponents of Cl, the first component of human serum complement, Biochem. J. 163: 219.
Sissons, J. G. P., Peters, D. K., Gwyn Williams, D., and Boulton-Jones, M. J., 1974, Skin lesions, angio-oedema and hypocomplementaemia, Lancet 2: 1350.
Sissons, J. G. P., West, R. J., Fallows, J., Williams, D. G., Boucher, B. J., Amos, N., and Peters, D. K., 1976, The complement abnormalities of lipodystrophy, N. Engl. J. Med. 294: 461.
Snyderman, R., Gewurz, H., and Mergenhagen, S. E., 1968, Interactions of the complement system with endotoxic lipopolysaccharide generation of a factor chemotactic for polymorphonuclear leukocytes, J. Exp. Med. 128: 259.
Snyderman, R., Phillips, J. K., and Mergenhagen, S. E., 1970, Polymorphonuclear leukocyte chemotactic activity in rabbit and guinea pig serum treated with immune complexes. Evidence for C5a as the major chemotactic factor, Infect. Immunol. 1: 521.
Snyderman, R., Pike, M. C., Szatalowicz, V., and Meadows, L. M., 1977, A familial deficiency of the fifth component of complement, Clin. Res. 25: 368.
Soothill, J. F., and Harvey, B. A. M., 1976, Defective opsonization—a common immunity deficiency, Arch. Dis. Child. 51: 91.
Soter, N., Austen, K. F., and Gigli, I., 1974, Urticaria arthralgias as manifestation of necrotizing angiitis (vasculitis), J. Invest. Dermatol. 63: 483.
Soter. N. A., Mihm, M. C., Gigli, I., Dvorak, H. F., and Austen, K. F., 1976, Two distinct cellular patterns in cutaneous necrotizing angiitis, J. Invest. Dermatol. 66: 344.
Soter, N., Mihm, M. C., Dvorak, H. F., and Austen, K. F., 1978, Cutaneous necrotizing vasculitis: A sequential analysis of the morphological alterations occurring after mast cell degranulation in a patient with a unique syndrome, Clin. Exp. Immunol. 32: 46.
Siegelberg, H. L., and Götze, O., 1972, Conversion of C3 proactivator and activation of the alternate pathway of complement activation by different classes and subclasses of human immunoglobulin, Fed. Proc. 31: 2478.
Spiro, R. G., 1967, Studies on the renal glomerular basement membrane. Preparation and chemical composition, J. Biol. Chem. 242: 1915.
Spitzer, R. E., Vallota, E. H., Forristal, J., Sudora, E., Stitzel, A., Davis, N. C., and West, C. D., 1969, Serum C3 lytic system in patients with glomerulonephritis, Science 164: 436.
Stern, R., Fu, M. S., Fotino, M., Agnello, V., and Kunkel, H. G., 1976, Hereditary C2 deficiency association with skin lesions resembling the discoid lesions of systemic lupus erythematosus, Arthritis Rheum. 19: 517.
Stolfi, R. L., 1968, Immune lytic transformation: A state of irreversible damage generated as a result of the reaction of the eighth component in the guinea pig complement system, J. Immunol. 100: 46.
Stroud, R. M., Mayer, M. M., Miller, J. A., and McKenzie, A. T., 1966, C2ad, an inactive derivative of C2 released during decay of EAC4,2a, Immunochemistry 3: 163.
Sussman, M., Jones, J. H., Almeida, J. D., and Lachmann, P. J., 1973, Deficiency of the second component of complement associated with anaphylactoid purpura and presence of mycoplasm in the serum, Clin. Exp. Immunol. 14: 531.
Tagami, H., and Ofuji, S., 1977, Characterization of a leukotactic factor derived from psoriatic scale, Br. J. Dermatol. 97: 509.
Tamura, N., and Nelson, R. A., 1967, Three naturally occurring inhibitors of components of complement in guinea pig and rabbit serum, J. Immunol. 99: 582.
Taranta, A., and Franklin, E. C., 1961, Complement fixation by antibody fragments, Science 134: 1981.
Tarantino, A., Anelli, A., Costino, A., DeVecchio, A., Monti, G., and Massaro, L., 1978, Serum complement in essential mixed cryoglobulinemia, Clin. Exp. Immunol. 32: 77.
Theophilopoulos, A. N., Bokisch, V. A., and Dixon, F. J., 1974, Receptor for soluble C3 and C3b on human lymphoblastoid (Raji) cells: Properties and biological significance, J. Exp. Med. 139:669.
Thompson, R. A., and White, R. H. R., 1973, Partial lipodystrophy and hypocomplementaemic nephritis, Lancet 2: 679.
Tobelem, G., Wautier, L. J., and Peltier, A. P., 1974, C2 deficiency, platelet aggregation reduction and Lob-stein’s disease in the same family, Biomed. Exp. 21: 190.
Tomar, R. H., and Kolchins, D., 1972, Complement and coagulation: Serum i 1c/0 la in disseminated intravascular coagulation, Thromb. Diath. Haemorrh. 27: 389.
Traub, W., and Piez, K. A., 1971, The chemistry and structure of collagen, Adv. Protein Chem. 25: 243.
Valet, G., and Cooper, N. R., 1974a, Isolation and characterization of the proenzyme form of the Cls subunit of the first complement component, J. Immunol. 122: 339.
Valet, G., and Cooper, N. R., 1974b, Isolation of the proenzyme form of the Clr subunit of the first complement component, J. Immunol. 112: 1667.
Vallota, E. H., Götze, O., Spiegelborg, H. H., Forriscal, J., West, D. C., and Müller-Eberhard, H. J., 1974, A serum factor in chronic hypocomplementemic nephritis distinct from immunoglobulin and activating the alternative pathway of complement, J. Exp. Med. 139: 1249.
Vogt, W., Schmidt, G., Von Buttlar, B., and Dieminger, L., 1978, A new function of the activated third component of complement: Binding to C5, an essential step for C5 activation, Immunology 34: 29.
Wakefield, M. A., 1976, The interrelationship between hypocomplementaemia, partial lipodystrophy and mesangiocapillary glomerulonephritis, Postgrad. Med. J. 52: 651.
Weiler, J. M., Daha, M. R., Austen, K. F., and Fearon, D. T., 1976, Control of the amplification convertase of complement by the plasma protein 01H, Proc. Natl. Acad. Sci. USA 73: 3268.
Weiss, T. D., Tsai, Cheng, Baldassare, and Zuckner, J., 1978, Skin lesions in viral hepatitis: Histologic and immunofluorescent studies, Am. J. Med. 64: 269.
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.
Wemanbu, S. N. C., Turk, J. L., Waters, M. F. R., and Rees, R. J. W., 1969, Erythema nodosum leprosum: A clinical manifestation of the Arthus phenomenon, Lancet 2: 933.
Whaley, K., and Ruddy, S., 1976a, Modulation of C3b hemolytic activity by a plasma protein distinct from C3b inactivation, Science 193: 1011.
Whaley, K., and Ruddy, S., 1976b, Modulation of the alternative complement pathway by 01H globulin, J. Exp. Med. 144: 1147.
Wild, J., Zvaifler, N., Müller-Eberhard, H., and Wilson, C., 1974, Deficiency of the second component (C2) in a patient with discoid lupus erythematosus (DLE), J. Clin. Invest. 53: 84.
Williams, C. A., and Chase, M. W., 1977, Methods in Immunology and Immunochemistry, Vol. 4, p. 127, Academic Press, New York.
Williams, R. C., Jr., and Kunkel, H. G., 1992, Rheumatoid factor, complement, and conglutinin aberrations in patients with subacute bacterial endocarditis, J. Clin. Invest. 41: 666.
Wuepper, K. D., Bokisch, V. A., Müller-Eberhard, H. J., and Stoughton, R. B., 1972, Cutaneous response to human C3a anaphylatoxin in man, Clin. Exp. Immunol. 11: 13.
Zeek, P. M., 1953, Periarteritis nodosa and other forms of necrotizing angiitis, N. Eng. J. Med. 248: 764.
Ziccardi, R. G., and Cooper, N. R., 1978, Modulation of the antigenicity of CTr and CTs by C f INH, J. Immunol. 121: 2148.
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© 1981 Plenum Publishing Corporation
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Gigli, I. (1981). The Complement System. In: Safai, B., Good, R.A. (eds) Immunodermatology. Comprehensive Immunology, vol 7. Springer, Boston, MA. https://doi.org/10.1007/978-1-4615-7228-2_4
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