Article
Hemolytic uremic syndrome: a factor H mutation (E1172Stop) causes defective complement control at the surface of endothelial cells.
Journal of the American Society of Nephrology : JASN - 1 Feb 2007
Heinen Stefan, Józsi Mihály, Hartmann Andrea, Noris Marina, Remuzzi Giuseppe, Skerka Christine, Zipfel Peter F
Abstract excerpt
Defective complement regulation results in hemolytic uremic syndrome (HUS), a disease that is characterized by microangiopathy, thrombocytopenia, and acute renal failure and that causes endothelial cell damage. For characterization of how defective complement regulation relates to the pathophysiology, the role of the complement regulator factor H and also of a mutant factor H protein was studied on the surface of...
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