Article
A hybrid CFHR3-1 gene causes familial C3 glomerulopathy.
Journal of the American Society of Nephrology : JASN - 1 Jul 2012
Malik Talat H, Lavin Peter J, Goicoechea de Jorge Elena, Vernon Katherine A, Rose Kirsten L, Patel Mitali P, de Leeuw Marcel, Neary John J, Conlon Peter J, Winn Michelle P, Pickering Matthew C
Abstract excerpt
Controlled activation of the complement system, a key component of innate immunity, enables destruction of pathogens with minimal damage to host tissue. Complement factor H (CFH), which inhibits complement activation, and five CFH-related proteins (CFHR1-5) compose a family of structurally related molecules. Combined deletion of CFHR3 and CFHR1 is common and confers a protective effect in IgA nephropathy. Here,...
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