Excessive activation of the complement system in atypical hemolytic uremic syndrome: is it ready for prime time?

ABSTRACT: Complement factor I (CFI) mutations are implicated in the pathogenesis of atypical hemolytic uremic syndrome (aHUS). Nevertheless, there is evidence that CFI deficiency is a weak effector of aHUS. Bienaime et al. report that homozygous deletion of CFHR-1 in the RCA gene cluster of chromosome 1q is a major risk factor for poor outcome for patients with CFI mutations. The basic and clinical implications of the findings are further elaborated here.

SUBMITTER: Tsai HM 

PROVIDER: S-EPMC3153079 | biostudies-literature | 2010 Feb

REPOSITORIES: biostudies-literature