PMID: 7019379Jul 1, 1981Paper

Mechanism of action of the C4 nephritic factor. Deregulation of the classical pathway of C3 convertase

The Journal of Experimental Medicine
I GigliJ Leibowitch

Abstract

Three mechanisms that regulate the formation and function of the classical pathway C3 convertase (C4b2a) have been elucidated: (a) an intrinsic decay of the enzyme that is temperature dependent; (b) an extrinsic decay mediated by the effect of the serum protein C4b binding protein (C4-bp); and (c) inactivation of C4b by the proteolytic action of C4b/C3b inactivator (C4b/C3bINA), which cleaves that alpha' chain of C4b to yield C4d (alpha 2) and C4c (alpha 3, alpha 4, beta, and gamma chains). A fourth mechanism described here is based on the observation that the IgG fraction of the serum of certain patients with glomerulonephritis contains a protein termed C4 nephritic factor (NFc), which prevents the intrinsic decay of C4b2a. This protein, which prolongs the half-life of surface-bound C4b2a from 7.5 min to greater than 5 h, increases the use of C3 and C5. It also inhibits the decay produced by C4-bp by preventing the dissociation of C2a from the C4b2a complex. Additionally, the C2b/C3bINA alone, or in the presence of C4-bp, fails to cleave the alpha' chain of C4b in the surface-bound stabilized C4b2a complex. This protective property of NFc requires the presence of C2a, because C4b was not protected unless it was bound to C2a. T...Continue Reading

References

Jan 1, 1976·Scandinavian Journal of Immunology·R D SchreiberH J Müller-Eberhard
Nov 2, 1976·The Journal of Experimental Medicine·K Whaley, S Ruddy
Sep 1, 1977·Proceedings of the National Academy of Sciences of the United States of America·A E DavisC A Alper
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Feb 26, 1974·Biochemistry·G S David, R A Reisfeld

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Citations

Mar 27, 1990·Journal of Immunological Methods·J SeinoT Saito
Jan 1, 1985·Biotechnology Advances·N W Lem, B R Glick
Sep 3, 2004·The Journal of Clinical Investigation·V Michael Holers
Jun 25, 2013·Molecular Immunology·Marie-Agnès Dragon-DureyLeendert A Trouw
Jul 16, 2011·Molecular Immunology·Nina A DahaLeendert A Trouw
Dec 14, 2019·Clinical Reviews in Allergy & Immunology·Federica DefendiChantal Dumestre-Pérard
May 10, 2019·Frontiers in Immunology·Fernando CorvilloMargarita López-Trascasa
Apr 27, 2019·Frontiers in Immunology·Jill J HauerRichard J H Smith
Jun 28, 2018·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Marina NorisGiuseppe Remuzzi

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