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lüll Complement factor H and the hemolytic uremic syndrome Atkinson JP; Goodship THJ Exp Med 2007[Jun]; 204 (6): 1245-8Immune 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, glomerulonephritides, 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.|*Disease Models, Animal[MESH]|*Models, Immunological[MESH]|Animals[MESH]|Complement Factor H/genetics/immunology[MESH]|Complement Pathway, Alternative/*immunology[MESH]|Hemolytic-Uremic Syndrome/*genetics/immunology[MESH]|Humans[MESH]|Macular Degeneration/*genetics/immunology[MESH]|Mice[MESH] |