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PubMed · 17198855

Complement factor H.

M de la Fuente·M J Blanco·B Pazos·M I Fernández·A Carracedo·M Sánchez-Salorio·R M Coco·C Torrón·A M Gómez
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Complement Factor HGenotypeHumansMacular DegenerationOdds RatioPolymorphism, Single NucleotideRisk FactorsSpainLetter

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M de la Fuente, M J Blanco, B Pazos, M I Fernández, A Carracedo, M Sánchez-Salorio, R M Coco, C Torrón, A M Gómez. 2007. Complement factor H.. https://doi.org/10.1016/j.ophtha.2006.10.004

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The haemolytic uraemic syndrome (HUS) is characterized by the triad of thrombocytopenia, microangiopathic haemolytic anaemia and acute renal failure. HUS may be classified as either diarrhoeal-associated or non-diarrhoeal/atypical (aHUS). aHUS has recently been shown to be a disease of complement dysregulation, with 50% of cases involving the complement regulatory genes, factor H (CFH), membrane cofactor protein (MCP; CD46), and factor I (IF). However, incomplete penetrance of mutations in each of these genes is reported. This suggests that a precipitating event or trigger is required to unmask the complement regulatory deficiency. The reported precipitating events predominantly cause endothelial injury. Discovery of these mutations has revealed important genotype-phenotype correlations. MCP-HUS has a better prognosis and a better outcome after transplantation than either CFH-HUS or IF-HUS.

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The role of defective complement control in hemolytic uremic syndrome.

Atypical hemolytic uremic syndrome (HUS) is a severe disease that is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. Recent evidence has shown that defective complement activation and defective complement control is a cause of HUS. So far, mutations in single genes coding for the cofactor and complement regulator factor H, the membrane cofactor protein (MCP/CD46), the serine protease factor I, and autoantibodies to factor H have been linked to HUS. All of these proteins affect the same enzyme the alternative pathway convertase C3bBb. This article explains how alternative pathway activation proceeds and how defective control increases activation, which ultimately leads to endothelial cell damage.

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