Article
Antibody recognition of complement factor H reveals a flexible loop involved in atypical hemolytic uremic syndrome pathogenesis.
The Journal of biological chemistry - 1 Jun 2022
Yokoo Takanori, Tanabe Aki, Yoshida Yoko, Caaveiro Jose M M, Nakakido Makoto, Ikeda Yoichiro, Fujimura Yoshihiro, Matsumoto Masaneori, Entzminger Kevin, Maruyama Toshiaki, Okumura C J, Nangaku Masaomi, Tsumoto Kouhei
Abstract excerpt
Atypical hemolytic uremic syndrome (aHUS) is a disease associated with dysregulation of the immune complement system, especially of the alternative pathway (AP). Complement factor H (CFH), consisting of 20 domains called complement control protein (CCP1-20), downregulates the AP as a cofactor for mediating C3 inactivation by complement factor I. However, anomalies related to CFH are known to cause excessive...
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