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Lookup NU author(s): Professor Tim Goodship
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 © The Rockefeller University Press.
Author(s): Atkinson JP, Goodship THJ
Publication type: Review
Publication status: Published
Journal: Journal of Experimental Medicine
Year: 2007
Volume: 204
Issue: 6
Pages: 1245-1248
Print publication date: 11/06/2007
ISSN (print): 0022-1007
ISSN (electronic): 1540-9538
URL: http://dx.doi.org/10.1084/jem.20070664
DOI: 10.1084/jem.20070664
PubMed id: 17548524
