The development of atypical hemolytic uremic syndrome depends on complement C5
EG de Jorge, P Macor… - Journal of the …, 2011 - journals.lww.com
Gene variants in the alternative pathway of the complement system strongly associate with
atypical hemolytic uremic syndrome (aHUS), presumably by predisposing to increased
complement activation within the kidney. Complement factor H (CFH) is the major regulator
of complement activation through the alternative pathway. Factor H-deficient mice
transgenically expressing a mutant CFH protein (Cfh−/−. FHΔ16–20) that functionally mimics
the CFH mutations reported in aHUS patients spontaneously develop thrombotic …
atypical hemolytic uremic syndrome (aHUS), presumably by predisposing to increased
complement activation within the kidney. Complement factor H (CFH) is the major regulator
of complement activation through the alternative pathway. Factor H-deficient mice
transgenically expressing a mutant CFH protein (Cfh−/−. FHΔ16–20) that functionally mimics
the CFH mutations reported in aHUS patients spontaneously develop thrombotic …