Restoration by purified C3b inactivator of complement-mediated function in vivo in a patient with C3b inactivator deficiency.
Open Access
- 1 March 1975
- journal article
- Published by American Society for Clinical Investigation in Journal of Clinical Investigation
- Vol. 55 (3) , 668-672
- https://doi.org/10.1172/jci107975
Abstract
In a patient with lifelong increased susceptibility to infection and multiple abnormalities in complement-mediated functions, the infusion of normal plasma had been seen to produce a prolonged partial correction of serum abnormalities. It was subsequently shown that the patient was genetically deficient in the C3b inactivator and that immunochemical depletion of C3b inactivator from normal serum resulted in abnormalities similar to those found in the patient's serum, including alternative pathway C3 activation. Highly purified C3b inactivator was obtained from the euglobulin fraction of normal human serum, sterilized by filtration, and infused intravenously. Partial or complete correction of almost all the known serum abnormalities was obtained. C3b almost disappeared from the serum within 4-5 h, as did Factor C activity. Native C3, C5, and serum hemolytic activity rose to normal or near-normal levels over 4 days and were sustained for another week. Factor B, properdin, opsonic activity, and bactericidal activity reached a level at least two-five times that found before the infusion within 24 h and fell over the next 5 days. These observations prove the primary role of C3b inactivator deficiency in the patient's disease and demonstrate clearly the curcial role in vivo of C3b inactivator in modulating alternative pathway activity.Keywords
This publication has 15 references indexed in Scilit:
- Alternative Pathway for the Activation of Complement in Human Serum. Formation and Composition of the Complex with Cobra Venom Factor that Cleaves the Third Component of ComplementBiological Chemistry, 1974
- Further studies on the C3b inactivator or conglutinogen activating factor (KAF)Immunochemistry, 1973
- Evaluation of Opsonic and Leukocyte Function With a Spectrophotometric Test in Patients With Infection and With Phagocytic DisordersBlood, 1973
- The alternate pathway of complement activation. The role of C3 and its inactivator (KAF).1973
- THE RELATIONSHIP OF GLYCINE-RICH ß-GLYCOPROTEIN TO FACTOR B IN THE PROPERDIN SYSTEM AND TO THE COBRA FACTOR-BINDING PROTEIN OF HUMAN SERUMThe Journal of Experimental Medicine, 1973
- FORMATION AND FUNCTION OF A COMPLEX OF THE C3 PROACTIVATOR WITH A PROTEIN FROM COBRA VENOMThe Journal of Experimental Medicine, 1973
- Inactivator of the Third Component of Complement as an Inhibitor in the Properdin PathwayProceedings of the National Academy of Sciences, 1972
- Deficiency of C3 Inactivator in ManThe Journal of Immunology, 1971
- Increased Susceptibility to Infection Associated with Abnormalities of Complement-Mediated Functions and of the Third Component of Complement (C3)New England Journal of Medicine, 1970
- Quantitative estimation of proteins by electrophoresis in agarose gel containing antibodiesAnalytical Biochemistry, 1966