Combination of factor H mutation and properdin deficiency causes severe C3 glomerulonephritis

AM Lesher, L Zhou, Y Kimura, S Sato… - Journal of the …, 2013 - journals.lww.com
Factor H (fH) and properdin both modulate complement; however, fH inhibits activation, and
properdin promotes activation of the alternative pathway of complement. Mutations in fH
associate with several human kidney diseases, but whether inhibiting properdin would be
beneficial in these diseases is unknown. Here, we found that either genetic or
pharmacological blockade of properdin, which we expected to be therapeutic, converted the
mild C3 GN of an fH-mutant mouse to a lethal C3 GN with features of human dense deposit …