Article
The solution structure of the complement deregulator FHR5 reveals a compact dimer and provides new insights into CFHR5 nephropathy.
The Journal of biological chemistry - 27 Nov 2020
Kadkhodayi-Kholghi Nilufar, Bhatt Jayesh S, Gor Jayesh, McDermott Lindsay C, Gale Daniel P, Perkins Stephen J
Abstract excerpt
The human complement Factor H-related 5 protein (FHR5) antagonizes the main circulating complement regulator Factor H, resulting in the deregulation of complement activation. FHR5 normally contains nine short complement regulator (SCR) domains, but a FHR5 mutant has been identified with a duplicated N-terminal SCR-1/2 domain pair that causes CFHR5 nephropathy. To understand how this duplication causes disease, we...
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