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The Complement System

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Biological Functions of Proteinases

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Abstract

The complement system (C) is composed of group of plasma proteins which under certain conditions react with each other sequentially, resulting in the formation of highly specialized proteases. By their action multimolecular complexes are assembled, and when these events occur on the surface of some cells, the membrane function is impaired and the cell will lyse. Fragments and complexes appearing on activation of the complement system participate in other important biological activities such as chemotaxis, opsonization and in histamin release from mast cells and basophilic granulocytes.

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References

  • Amos N, Sissons JPG, Peters DK (1977) Binding of nephritic factor by anti-IgG and protein A. Pathol Biol 25: 390

    Google Scholar 

  • Arroyave CM/ Müller-Eberhard HJ (1973) Interactions between human C5, C6 and C7 and their functional significance in complement dependent cytolysis. J Immunol 111: 536–545

    PubMed  CAS  Google Scholar 

  • Berglund K, Johansson BG, Laureil A-B, Sjöholm AG, Sturfelt G (1979) Immune complexes, complement activation and Cl subcomponent complexes in rheumatoid arthritis. Effects of treatment with podophyllotoxin derivatives and cyclophosphamide. In preparation

    Google Scholar 

  • Bianco C, Nussenzweig V (1977) Complement receptors. In: Porter RR, Ada GL (eds) Contemporary topics in molecular immunology, vol 6. Plenum Press, New York, p 145–176

    Google Scholar 

  • Bokisch VA, Müller-Eberhard HJ (1970) Anaphylatoxin inactivator of human plasma: its isolation and characterization as a carboxypeptidase. J Clin Invest 49: 2427–2436

    Article  PubMed  CAS  Google Scholar 

  • Bokisch VA, Müller-Eberhard HJ, Cochrane CG (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: 1109–1130

    Google Scholar 

  • Bokisch VA, Dierich MP, Müller-Eberhard HJ (1975) Third complement component (C3): Structural properties in relation to functions. Proc Natl Acad Sci USA 72: 1989–1993

    Google Scholar 

  • Cooper NR (1975) Isolation and analysis of the mechanism of action of an inactivator of C4b in normal human serum. J Exp Med 141: 890–903

    PubMed  CAS  Google Scholar 

  • Daha MR, Fearon DT, Austen KF (1978) Heterogeneity, polypeptide chain composition and antigenic reactivity of C3 nephritic factor. J Immunol 120: 1389–1394

    PubMed  CAS  Google Scholar 

  • Davis AE, Ziegler JB, Gelfand EM, Rosen FS, Alper CA (1977) Heterogeneity of nephritic factor and its identification as an immunoglobulin. Proc Natl Acad Sci USA 74: 3980–3983

    Article  PubMed  CAS  Google Scholar 

  • Donaldson VH, Evans RR (1963) A biochemical abnormality in hereditary angioneurotic edema. Absence of serum inhibitor of C’1 esterase. Am J Med 35: 37–44

    Article  PubMed  CAS  Google Scholar 

  • Donaldson VH, Rosen FS (1964) Action of complement in hereditary angioneurotic edema: the role of C’1 esterase. J Clin Invest 43: 2204–2213

    Article  PubMed  CAS  Google Scholar 

  • Donaldson VH, Merler E, Rosen FS, Kretschmer KW, Lepow IH (1970) A polypeptide kinin in hereditary angioneurotic edema plasma: role of complement in its formation. J Lab Clin Med 76: 936

    Google Scholar 

  • Fearon DT (1978) Regulation by membrane sialic acid of 3lH-dependent decay-dissociation of amplification C3 convertase of the alternative complement pathway. Proc Natl Acad Sci USA 75: 1971–1975

    Article  PubMed  CAS  Google Scholar 

  • Fearon DT, Austen KF (1978) Immunochemistry of the classical and alternative pathways of complement. In: Glynn LE, Steward MW (eds) Immunochemistry: an advanced textbook. John Wiley Sons Ltd, New York, p 365–397

    Google Scholar 

  • Fearon DT, Austen KF, Ruddy S (1974) Properdin factor D. II. Activation to D by properdin. J Exp Med 140: 426–436

    Google Scholar 

  • Forbes CD, Pensky J, Ratnoff OD (1970) Inhibition of activated Hageman factor and activated plasma thromboplastin antecedent by purified serum Cl inactivator. J Lab Clin Med 76: 809–815

    PubMed  CAS  Google Scholar 

  • Fujita T, Gigli I, Nussenzweig V (1978) Human C4-binding protein. II. Role in proteolysis of C4b by C3b inactivator. J Exp Med 148: 1044–1051

    Article  PubMed  CAS  Google Scholar 

  • Gewürz H, Lint TF (1977) Alternative modes and pathways of complement activation. In: Day NK, Good RA (eds) Comprehensive immunology. II. Biological amplification systems of immunology. Plenum Press, New York, p 17–45

    Google Scholar 

  • Götze O (1975) Proteases of the properdin system. In: Reich E, Rifkin DF, Shaw E (eds) Proteases and biological control. Cold Spring Harbor Laboratory, New York, p 255–272

    Google Scholar 

  • Harpe1 PC, Cooper NR (1975) Studies on human plasma Cl inactivator—enzyme interaction. J Clin Invest 55: 593–604

    Article  PubMed  CAS  Google Scholar 

  • Johnson U (1974) Properdin acting as a C3 convertase. Acta Pathol Microbiol Scand Sect B 83: 914–916

    Google Scholar 

  • Johnson U (1978) The role of Cls, Clr and properdin in the initiation of the C3 feed-back mechanism of the complement system. Acta Pathol Microbiol Scand Sect C 86: 73–78

    CAS  Google Scholar 

  • Johnson U, Ohlsson K, Olsson I (1976) Effects of granulocyte neutral proteases on complement components. Scand J Immunol 5: 421–426

    Article  PubMed  CAS  Google Scholar 

  • Kaplan MH, Volanakis JE (1974) Interaction of C-reactive protein complexes with the complement system. I. Consumption of human complement associated with the reaction of C-reactive protein with pneumococcal C-polysaccharide and with choline phosphatide, lecithine and sphingomyelin. J Immunol 112: 2135–2147

    Google Scholar 

  • Kolb WP, Müller-Eberhard HJ (1975) The membrane attack mechanism of complement. Isolation and subunit composition of the C5b-9 complex. J Exp Med 141: 724–735

    PubMed  CAS  Google Scholar 

  • Lachmann PJ, Müller-Eberhard HJ (1968) The demonstration in human serum of “conglutinogen activating factor” and its effect on the third component of complement. J Immunol 100: 691–698

    PubMed  CAS  Google Scholar 

  • Landerman NS, Webster ME, Becker EL, Ratcliff HE (1972) Hereditary angioneurotic edema. II. Deficiency of inhibitor for serum globulin permeability factor and/or plasma kallikrein. J’ Allergy 33: 330–341

    Google Scholar 

  • Laurell A-B, Martensson U (1971) Cl inactivator protein complexed with albumin in plasma from a patient with angioneurotic edema. Eur J Immunol 1: 146–149

    Article  PubMed  CAS  Google Scholar 

  • Laurell A-B, Martensson U, Sjöholm AG (1976) Cl subcomponent complexes in normal and pathological sera studied by crossed Immunoelectrophoresis. Acta Pathol Microbiol Scand Sect C 84: 455–464

    Google Scholar 

  • Laurell A-B, Martensson U, Sjöholm AG (1977) Studies of Cl subcomponents in chronic urticaria and angioedema. Int Arch Allergy Appl Immunol 54: 4 34–442

    Google Scholar 

  • Laurell A-B, Johnson U, Martensson U, Sjöholm AG (1978) Formation of complexes composed of Clr, Cls and Cl inactivator in human serum on activation of Cl. Acta Pathol Microbiol Scand Sect C 86: 299–306

    Google Scholar 

  • Lepow IH, Naff GB, Todd EW, Pensky J, Hinz CF Jr (1963) Chromatographic resolution of the first component of complement into three activities. J Exp Med 117: 983–1008

    Article  PubMed  CAS  Google Scholar 

  • Medicus RG, Götze O, Müller-Eberhard HJ (1976) The serine protease nature of the C3 and C5 convertases of the classical and alternative complement pathways. Scand J Immunol 5: 1049–1055

    Article  PubMed  CAS  Google Scholar 

  • Müller-Eberhard HJ (1975) The complement system. In: Putnam FW (ed) The plasma proteins, vol I, 2nd edn, p 394–432

    Google Scholar 

  • Müller-Eberhard HJ, Polley MJ, Calcott MA (1967) Formation and functional significance of a molecular complex derived from the second and fourth component of com piement. J Exp Med 125: 359–380

    Article  PubMed  Google Scholar 

  • Nagasawa S, Stroud RM (1977) Mechanism of action of the C3b inactivator: requirement for a high molecular weight co-factor (C3b-C4b INA co-factor) and production of a new C3b derivative ( C3b’ ). Immunochemistry 14: 749–756

    Google Scholar 

  • Nicol PAE, Lachmann PJ (1973) The alternate pathway of complement activation. The role of C3 and its inactivator ( KAF ). Immunology 24: 259–275

    Google Scholar 

  • Nydegger UE, Fearon DT, Austen KF (1978) Autosomal locus regulates inverse relationship between sialic acid content and capacity of mouse erythrocytes to activate human alternative complement pathway. Proc Natl Acad Sci USA 75: 6078–6082

    Article  PubMed  CAS  Google Scholar 

  • Pangburn MK, Müller-Eberhard HJ (1978) Complement C3 convertase: Cell surface restriction of 31H control and generation of restriction on neuraminidase treated cells. Proc Natl Acad Sci USA 75: 2416–2420

    Article  PubMed  CAS  Google Scholar 

  • Pangburn MK, Schreiber RD, Müller-Eberhard HJ (1977) Human complement C3b inactivator: isolation, characterization and demonstration of an absolute requirement for the serum 31H for cleavage of C3b and C4b in solution. J Exp Med 146: 257–270

    Article  PubMed  CAS  Google Scholar 

  • Pensky J, Levy LR, Lepow IH (1961) Partial purification of a serum inhibitor of Cl esterase. J Biol Chem 236: 1674–1679

    PubMed  CAS  Google Scholar 

  • Podack ER, Kolb NP, Müller-Eberhard HJ (1978) The C5b-6 complex: formation, isolation and inhibition of its activity by lipoproteins and the S-protein of human serum. J Immunol 120: 1841–1848

    PubMed  CAS  Google Scholar 

  • Ratnoff OD, Naff GB (1967) The conversion of C’ls to C’l esterase by plasmin and trypsin. J Exp Med 125: 337–358

    Article  PubMed  CAS  Google Scholar 

  • Ratnoff OD, Pensky J, Ogston D, Naff GB (1969) The inhibition of plasmin, plasma kallikrein, plasma permeability factor and the Clr subcomponent of the first complement component by serum esterase inhibitor. J Exp Med 129: 315–331

    Article  PubMed  CAS  Google Scholar 

  • Rosen FS, Charache P, Pensky J, Donaldson VH (1965) Hereditary angioneurotic edema: two genetic variants. Science 148: 957–958

    Article  PubMed  CAS  Google Scholar 

  • Rosen FS, Alper CA, Pensky J, Klemperer MR, Donaldson VH (1971) Genetically determined heterogeneity of the Cl esterase inhibitor in patients with hereditary angioneurotic edema. J Clin Invest 50: 2143–2149

    Article  PubMed  CAS  Google Scholar 

  • Ruddy S, Austen KF (1971) C3b inactivator of man. II. Fragments produced by C3b inactivator cleavage of cellbound or fluid phase C3b. J Immunol 107: 742–750

    PubMed  CAS  Google Scholar 

  • Sakai K, Stroud RM (1973) Purification, molecular properties and activation of Cl proesterase, Cls. J Immunol 110: 1010–1020

    Google Scholar 

  • Sakai K, Stroud RM (1974) The activation of Cls with purified Clr. Immunochemistry 11: 191–196

    Article  PubMed  CAS  Google Scholar 

  • Schreiber RD, Pangburn MK, Lesavre PH, Miiller-Eberhard HJ (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: 3948–3952

    Article  PubMed  CAS  Google Scholar 

  • Shiraishi S, Stroud RM (1975) Cleavage products of C4b produced by enzymes in human serum. Immunochemistry 12: 935–939

    Article  PubMed  CAS  Google Scholar 

  • Sjoholm AG (1979) Complement components and complement activation in acute poststreptococcal glomerulonephritis. Int Arch Allergy Appl Immun 58: 241–250

    Article  Google Scholar 

  • Spitzer RE, Vallota EH, Forristal J, Sudora E, Stitzel A, Davis NC, West CD (1969) Serum C’3 lytic system in patients with glomerulonephritis. Science, New York 164: 436–437

    Google Scholar 

  • Taylor JC, Crawford IP, Hugli TE (1977) Limited degradation of the third component (C3) of human complement by human leukocyte elastase (HLE): partial characterization of C3 fragments. Biochemistry 16: 3390–3396

    Article  PubMed  CAS  Google Scholar 

  • Vallota EH, Miiller-Eberhard HJ (1973) Formation of C3a and C5a anaphylatoxins in whole human serum after inhibition of the anaphylatoxin inactivator. J Exp Med 137: 1109–1123

    Article  PubMed  CAS  Google Scholar 

  • Vogt W, Schmidt G, Lynen R, Dieminger L (1975) Cleavage of the third complement component (C3) and generation of the spasmogenic peptide, C3a, in human serum via the properdin pathway: demonstration of inhibitory as well as enhancing effects of epsilon-amino-caproic acid. J Immunol 114: 671–677

    PubMed  CAS  Google Scholar 

  • Ward PA (1967) A plasmin-split fragment of C’3 as a new chemotactic factor. J Exp Med 126: 189–206

    Article  PubMed  CAS  Google Scholar 

  • Weiler JM, Daha MR, Austen KF, Fearon DT (1976) Control of the amplification convertase of complement by the plasma protein 31H. Proc Natl Acad Sci USA 73: 3268–3272

    Article  PubMed  CAS  Google Scholar 

  • Ziccardi RJ, Cooper NR (1976) Activation of Clr by proteolytic cleavage. J Immunol 5: 1049–1053

    Google Scholar 

  • Ziccardi RJ, Cooper NR (1978a) Demonstration and quantitation of activation of the first component of complement in human serum. J Exp Med 147: 385–395

    Article  PubMed  CAS  Google Scholar 

  • Ziccardi RJ, Cooper NR (1978b) Modulation of the antigenicity of clr and els by Cl inactivator. J’ Immunol 121: 2148–2152

    PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Institute of Medical Microbiology, Department of Immunology, University of Lund, Sölvegatan 23, S-223 62, Lund, Sweden

    A.-B. Laurell

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  1. A.-B. Laurell
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Editors and Affiliations

  1. Biochemisches Institut, Albert-Ludwigs-Universität, Hermann-Herder-Straße 7, 7800, Freiburg, Germany

    Helmut Holzer

  2. Lehrstuhl für Biochemie Fakultät für Chemie, Universität Bielefeld, Universitätsstraße, 4800, Bielefeld, Germany

    Harald Tschesche

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© 1979 Springer-Verlag Berlin Heidelberg

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Laurell, AB. (1979). The Complement System. In: Holzer, H., Tschesche, H. (eds) Biological Functions of Proteinases. Colloquium der Gesellschaft für Biologische Chemie 26.–28. April 1979 in Mosbach/Baden, vol 30. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-81395-5_20

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  • DOI: https://doi.org/10.1007/978-3-642-81395-5_20

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-81397-9

  • Online ISBN: 978-3-642-81395-5

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