Article
Combination of factor H mutation and properdin deficiency causes severe C3 glomerulonephritis.
Journal of the American Society of Nephrology : JASN - 1 Jan 2013
Lesher Allison M, Zhou Lin, Kimura Yuko, Sato Sayaka, Gullipalli Damodar, Herbert Andrew P, Barlow Paul N, Eberhardt Hannes U, Skerka Christina, Zipfel Peter F, Hamano Takayuki, Miwa Takashi, Tung Kenneth S, Song Wen-Chao
Abstract excerpt
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...
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