[HTML][HTML] Partial complement factor H deficiency associates with C3 glomerulopathy and thrombotic microangiopathy

KA Vernon, MM Ruseva, HT Cook, M Botto… - Journal of the …, 2016 - journals.lww.com
The complement–mediated renal diseases C3 glomerulopathy (C3G) and atypical hemolytic
uremic syndrome (aHUS) strongly associate with inherited and acquired abnormalities in the
regulation of the complement alternative pathway (AP). The major negative regulator of the
AP is the plasma protein complement factor H (FH). Abnormalities in FH result in
uncontrolled activation of C3 through the AP and associate with susceptibility to both C3G
and aHUS. Although previously developed FH–deficient animal models have provided …