Complement factor H and the hemolytic uremic syndrome

John P. Atkinson, Timothy H.J. Goodship

Research output: Contribution to journalReview articlepeer-review

59 Scopus citations

Abstract

Immune recognition is coupled to powerful proinflammatory effector pathways that must be tightly regulated. The ancient alternative pathway of complement activation is one such proinflammatory pathway. Genetic susceptibility factors have been identified in both regulators and activating components of the alternative pathway that are associated with thrombotic microangiopathies, glomer ulonephritides, and chronic conditions featuring debris deposition. These observations indicate that excessive alternative pathway activation promotes thrombosis in the microvasculature and tissue damage during debris accumulation. Intriguingly, distinct genetic changes in factor H (FH), a key regulator of the alternative pathway, are associated with hemolytic uremic syndrome (HUS), membranoproliferative glomerulonephritis (dense deposit disease), or age-related macular degeneration (AMD). A mouse model of HUS designed to mirror human mutations in FH has now been developed, providing new understanding of the molecular pathogenesis of complement-related endothelial disorders. JEM

Original languageEnglish
Pages (from-to)1245-1248
Number of pages4
JournalJournal of Experimental Medicine
Volume204
Issue number6
DOIs
StatePublished - Jun 11 2007

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