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Research Article Free access | 10.1172/JCI107254

Hypocomplementemia of Membranoproliferative Nephritis DEPENDENCE OF THE NEPHRITIC FACTOR REACTION ON PROPERDIN FACTOR B

Edward J. Ruley, Judith Forristal, Neil C. Davis, Cynthia Andres, and Clark D. West

Children's Hospital Research Foundation and the Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229

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Children's Hospital Research Foundation and the Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229

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Children's Hospital Research Foundation and the Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229

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Children's Hospital Research Foundation and the Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229

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Children's Hospital Research Foundation and the Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio 45229

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Published April 1, 1973 - More info

Published in Volume 52, Issue 4 on April 1, 1973
J Clin Invest. 1973;52(4):896–904. https://doi.org/10.1172/JCI107254.
© 1973 The American Society for Clinical Investigation
Published April 1, 1973 - Version history
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Abstract

Membranoproliferative nephritis in children is frequently associated with a hypocomplementemia produced at least in part by C3 breakdown mediated by a circulating anticomplementary factor known as C3 nephritic factor (C3NeF). C3 breakdown by this factor in vitro requires the presence of a pseudoglobulin cofactor and magnesium. The present study provides evidence that properdin factor B (C3 proactivator) is activated in the nephritic factor reaction and is the direct mediator of C3 breakdown by C3NeF. Depletion of factor B from mixtures of normal human serum (NHS) and plasma from a patient with membranoproliferative nephritis (MPP), either by heating or by immune equivalence absorption, blocks C3 breakdown by C3NeF. Addition of purified factor B to these mixtures restores the anticomplementary effect. When purified factor B is added to mixtures of MPP and purified C3, breakdown also occurs. Associated with the C3 breakdown is a change in the electrophoretic mobility of factor B from the beta to the gamma position, a shift which has been associated with cleavage activation of the molecule. Further, serum factor B levels are often low in patients with membranoproliferative nephritis and bear a rough inverse correlation with C3NeF levels. It thus appears that factor B is the previously described heat-labile C3NeF cofactor. Whether the C3NeF reaction proceeds via a pathway comparable to that activated by the cobra venom factor or via that activated by zymosan or inulin cannot be determined from the present data.

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