Atypical hemolytic uremic syndrome (aHUS) is a disease of complement dysregulation. In
approximately 50% of patients, mutations have been described in the genes encoding the …

The pathogenesis of atypical hemolytic uremic syndrome (aHUS) is strongly linked to
dysregulation of the alternative pathway of the complement system. Mutations in …

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 …

Central to the pathogenesis of atypical hemolytic uremic syndrome (aHUS) is over-activation
of the alternative pathway of complement. Following the initial discovery of mutations in the …

Over the past decade, atypical hemolytic uremic syndrome (aHUS) has been demonstrated
to be a disorder of the regulation of the complement alternative pathway. Among …

Mutations in the plasma complement regulator factor H (CFH) and the transmembrane
complement regulator membrane co-factor protein (MCP) have been shown to predispose to …

Genetic studies have demonstrated the involvement of the complement regulator factor H in
nondiarrheal, nonverocytotoxin (ie, atypical) cases of hemolytic uremic syndrome. Different …

Hemolytic uremic syndrome (HUS) is an important cause of acute renal failure in children.
Mutations in one or more genes encoding complement-regulatory proteins have been …

Mutations in the gene encoding complement factor H (CFH) that alter the C3b/polyanions-
binding site in the C-terminal region impair the capacity of factor H to protect host cells …

Several recent studies have established an association between abnormalities of
complement factor H (FH) and the development of hemolytic uremic syndrome (HUS). To …