Abstract
Complement factor H related protein 5 (CFHR5) nephropathy is a monogenic disorder of complement regulation that is endemic in Cyprus. The disease is characterised by haematuria, C3 glomerulonephritis and kidney failure. Its identification suggests a role for the CFHR5 protein in the regulation of complement in the kidney. In this review, we discuss how studying CFHR5 nephropathy can contribute to our understanding of the role of complement in kidney diseases such as dense deposit disease, C3 glomerulonephritis and atypical haemolytic uraemic syndrome.
Highlights
Complement factor H related protein 5 (CFHR5) nephropathy is a recently recognised kidney disease in which a heterozygous mutation in the CFHR5 gene is associated with autosomal dominant inheritance of glomerulonephritis and kidney failure (Gale et al, 2010)
We first discuss how the features of CFHR5 nephropathy relate to our current understanding of complement regulation and its role in kidney disease
We discuss the role of complement in membranoproliferative glomerulonephritis (MPGN), dense deposit disease (DDD), C3 glomerulonephritis and atypical haemolytic uraemic syndrome
Summary
Complement factor H related protein 5 (CFHR5) nephropathy is a monogenic disorder of complement regulation that is endemic in Cyprus. The disease is characterised by haematuria, C3 glomerulonephritis and kidney failure. Its identification suggests a role for the CFHR5 protein in the regulation of complement in the kidney. We discuss how studying CFHR5 nephropathy can contribute to our understanding of the role of complement in kidney diseases such as dense deposit disease, C3 glomerulonephritis and atypical haemolytic uraemic syndrome
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