J Biol Chem 2022 Apr 19:101962. Epub 2022 Apr 19.
Department of Chemistry and Biotechnology, School of Engineering, The University of Tokyo, 7-3-1, Hongo, Bunkyo-ku, Tokyo 113-8656, Japan; Department of Bioengineering, School of Engineering, The University of Tokyo, 7-3-1, Hongo, Bunkyo-ku, Tokyo, 113-8656, Japan; The institute of Medical Science, The University of Tokyo, 4-6-1, Shirokanedai, Minato-ku, Tokyo, 108-8639, Japan. Electronic address:
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 CCP1-20, downregulates the AP as a cofactor for mediating C3 inactivation by complement factor I (CFI). However, anomalies related to CFH are known to cause excessive complement activation and cytotoxicity. Read More