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, we report an
autosomal dominant complement-mediated GN associated with abnormal increases in copy …