Article
The major autoantibody epitope on factor H in atypical hemolytic uremic syndrome is structurally different from its homologous site in factor H-related protein 1, supporting a novel model for induction of autoimmunity in this disease.
The Journal of biological chemistry - 10 Apr 2015
Bhattacharjee Arnab, Reuter Stefanie, Trojnár Eszter, Kolodziejczyk Robert, Seeberger Harald, Hyvärinen Satu, Uzonyi Barbara, Szilágyi Ágnes, Prohászka Zoltán, Goldman Adrian, Józsi Mihály, Jokiranta T Sakari
Abstract excerpt
Atypical hemolytic uremic syndrome (aHUS) is characterized by complement attack against host cells due to mutations in complement proteins or autoantibodies against complement factor H (CFH). It is unknown why nearly all patients with autoimmune aHUS lack CFHR1 (CFH-related protein-1). These patients have autoantibodies against CFH domains 19 and 20 (CFH19-20), which are nearly identical to CFHR1 domains 4 and 5...
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