Factor H autoantibodies have been reported in approximately 10% of patients with atypical
hemolytic uremic syndrome (aHUS) and are associated with deficiency of factor H–related …

Atypical hemolytic uremic syndrome (aHUS) is a severe renal disease that is associated with
defective complement regulation caused by multiple factors. We previously described the …

The atypical form of the kidney disease hemolytic uremic syndrome (aHUS) is associated
with defective complement regulation. In addition to mutations in complement regulators …

Genomic disorders affecting the genes encoding factor H (fH) and the 5 factor H related
proteins have been described in association with atypical hemolytic uremic syndrome …

Haemolytic uraemic syndrome (HUS) is a severe disease with renal failure,
microangiopathic anemia and thrombocytopenia. Several mechanisms leading to HUS have …

Results Homozygous deletion in CFHR1 was detected in 32% of the patients with aHUS
tested, compared with 2.5% of controls (P< 0.001). CFH antibodies were present in 25% of …

Although genetic defect of complement factor H (CFH) is a common cause of atypical
hemolytic uremic syndrome (aHUS), development of autoantibodies to CFH (CFH-Ab) is …

The factor H–related protein family (CFHR) is a group of minor plasma proteins genetically
and structurally related to complement factor H (fH). Notably, deficiency of CFHR1/CFHR3 …

Factor I (FI) is the major complement inhibitor that degrades C3b and C4b in the presence of
cofactors such as factor H (FH) and membrane cofactor protein (MCP). Recently, mutations …

Background: Deletion of the complement factor H related 1 (CFHR1) gene is a consequence
of non-allelic homologous recombination and has been reported to be more frequent in …