PARTIAL PROPERDIN DEFICIENCY
- 1 January 1980
- journal article
- research article
- Vol. 96 (4) , 633-639
Abstract
The action of properdin in supporting complement C consumption was investigated in the serum from 2 [human] individuals with partial properdin deficiency. These studies are in agreement with those in which serum was artificially depleted of properdin in showing that serum deficient in properdin results in less C3 consumption in the presence of activators of the alternative and classical C pathways. Properdin deficiency did not lessen C3 consumption by C3NeF [C3 nephritic factor] or CoF [cobra venom factor], confirming that properdin is not necessary for the action of these substances. Partial properdin deficiency apparently is innocuous; complete absence of properdin may severely limit one''s ability to amplify C activation.This publication has 1 reference indexed in Scilit:
- 46,XY/46,XY,21q- mosaicism in an infant with neutropenia and properdin deficiency.Journal of Medical Genetics, 1976