Mechanism of action of anti-C1-inhibitor autoantibodies: Prevention of the formation of stable C1s-C1-inh complexes

Molecular Medicine

Volumn 4, Issue 2, 1998, Pages 119-128

  He, Shiping ^a,c   Sim, Robert B ^b   Whaley, Keith ^a  

^a UNIVERSITY OF LEICESTER   (United Kingdom)

^b UNIVERSITY OF OXFORD   (United Kingdom)

^c UNIVERSITY OF BIRMINGHAM   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

AUTOANTIBODY; COMPLEMENT COMPONENT C1S INHIBITOR;

AMINO ACID SEQUENCE; ARTICLE; CARBOXY TERMINAL SEQUENCE; COMPLEMENT ACTIVATION; COMPLEX FORMATION; COVALENT BOND; DRUG MECHANISM; ENZYME INHIBITION; ENZYME SUBSTRATE; HUMAN; HUMAN CELL; HYDROLYSIS; NEUTRON SCATTERING; PEPTIDE SYNTHESIS; PRIORITY JOURNAL;

EID: 0031946098     PISSN: 10761551     EISSN: None     Source Type: Journal    
DOI: 10.1007/bf03401735     Document Type: Article

References (29)

1. Davis, AE. (1988) Cl-inhibitor and hereditary angioneurotic edema. Annu. Rev. Immunol. 6: 595-628.
2. Perkins SJ, Smith KF, Amatayakul S, Ashford D, Rademacher TW, Dwek RA, Lachmann PJ, Harrison RA. (1990) The two-domain structure of the native and reaction centre cleaved forms of Cl-inhibitor of human complement by neutron scattering. J. Mol. Biol. 214: 751-768.
3. Ziccardi RJ. (1985) Demonstration of the interaction of native Cl with monomeric immunoglobulins and Cl-inhibitor. J. Immunol. 134: 2559-2563.
4. Sim RB, Arland GJ, Colomb MG. (1979) Cl-inhibitor dependent dissociation of human complement component Cl bound to immune complexes. Biochem. J. 179: 449-457.
5. Stein PE, Carrell RW. (1995) What dysfunctional serpins tell us about molecular mobility and disease. Structural Biol. 2: 96-113.
6. Geha RS, Quinti I, Ansten KF, Cicardi M, Sheffer A, Rosen FS. (1985) Acquired Cl-inhibitor deficiency associated with anti-idiotype antibody to monoclonal immunoglobulins. N. Engl. J. Med. 312: 534-540.
7. Jackson J, Sim RB, Whelan A, Feighery C. (1986) An IgG auloantibody which inactivates Cl-inhibitor. Nature 323: 722-724.
8. Alsenz J, Bork K, Loos M. (1987) Autoantibody-mediated acquired deficiency of Cl-inhibitor. N. Engl. J. Med. 316: 1360-1366.
9. He S, Tsang S, North J, Chohan N, Sim RB, Whaley K. (1996) Epitope mapping of Cl-inhibitor autoantibodies from patients with acquired Cl-inhibitor deficiency. J. Immunol. 154: 2009-2013.
10. Hudson L, Hay F. (1989) The basic techniques. In: Practical Immunology, 3rd ed. Blackwell Scientific Publications, Oxford, pp. 1-3.
11. Pilatte Y, Hammer CH, Frank MM, Fries LF. (1989) A new simplified procedure for Cl-inhibitor preparation: A novel use for Jacalin-agarose. J. Immunol. Methods 120: 37-43.
12. Sim RB. (1981) The human complement system serine proteases Clr and Cls and their proenzymes. Methods Enzymol. 80: 26-42.
13. Harrison RA. (1983) Human Cl-inhibitor: Improved isolation and preliminary structural characterisation. Biochemistry 22: 5001-5007.
14. Gigli I, Porter RR, Sim RB. (1976) The unactived form of the first component of human complement, Cl. Biochem J. 157: 541-548.
15. Markwell MAK. (1982) A new solid-state reagent to iodinate proteins. I. conditions for the efficient labelling of antiserum. Anal. Biochem. 125: 427-432.
16. Laemmli UK. (1970) Cleavage of structural proteins during the assembly of the head of the bacteriophage T4. Nature 227: 680-685.
17. Towbin H, Staehelin T, Gordon J. (1979) Electrophoretic transfer of proteins from polyacrylamide gel to nitrocellulose sheets: Procedure and some applications. Proc. Natl. Acad. Sci. U.S.A. 76: 4350-4354.
18. Wallace EM, Perkins SJ, Sim RB, Willis AC, Feighery C, Jackson J. (1997) Degradation of Cl-Inhibitor by Plasmin: Implications for the Control of Inflammatory Processes. Molec. Med. 3: 385-396.
19. Sim RB, Reboul A. (1981) Preparation and properties of human Cl-inhibitor. Methods Enzymol. 80: 43-53.
20. Cohen GH, Silverton EW, Davis DR. (1981) Refined crystal structure of γ-chymotrypsin at 1.9Å resolution. Comparison with other pancreatic serine proteases. J. Mol. Biol. 148: 449-479.
21. Birktoft JJ, Blow DM. (1972) Structure of crystalline α-chymotrypsin. V. The atomic structure of tosyl-α-chymotrypsin at 2Å resolution. J. Mol. Biol. 68: 187-240.
22. Fothergill J, Kemp G, Paton N, Carter P, Gray P. (1989) The structure of human Clr and Cls and their relationship to other serine proteases. Behring Inst. Mitt. 84: 72-79.
23. Mandle R, Baron C, Roux E, Sundal R, Gelfand J, Aulak K, Davis AE, Rosen FS, Bing D. (1994) Acquired Cl-inhibitor deficiency as a result of an autoantibody to the reactive centre region of Cl-inhibitor. J. Immunol. 152: 4680-4685.
24. Skriver K, Wikoff WR, Patston PA, Tauske F, Schapira M, Kaplan AP, Bock SC. (1991) Substrate properties of Cl-inhibitor Ma (alanine 434 → glutamic acid). J. Biol. Chem. 266: 9216-9221.
25. Davis AE, Aulak KS, Parad RB, Stecklein HP, Eldering E, Hack LE, Kramer J, Strunk RC, Rosen FS. (1992) Cl-inhibitor hinge region mutations produce dysfunction by different mechanisms. Nature Genet. 1: 354-358.
26. Jackson J, Sim RB, Whaley K, Feighery C. (1989) Autoantibody mediated cleavage of novel Cl-inhibitor by purified Clr, Cls and plasmin. J. Clin. Invest. 83: 698-707.
27. He S, Sim RB, Whaley K. (1997) A secondary Cls interaction site on Cl-inhibitor is essential for formation of a stable enzyme-inhibitor complex. FEBS Lett. 405: 42-46.
28. Ishizaki E, Yoshioka Y, Mori Y, Koyama J. (1976) Isolation of two forms of activated Cls, a subcomponent of the first component of rabbit complement. J. Biochem. 80: 1423-1427.
29. Mori Y, Ueda E, Takenchi T, Taniuchi S, Koyama J. (1980) Proteolytic cleavage of an activated subcomponent of the first component of rabbit complement, Cls. J. Biochem. 87: 1757-1763.