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Download 624-632 January 15, 2008
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Transcript
Membrane cofactor protein mutations in atypical hemolytic uremic syndrome (aHUS), fatal Stx-HUS, C3 glomerulonephritis, and the HELLP syndrome by Celia J. Fang, Veronique Fremeaux-Bacchi, M. Kathryn Liszewski, Gaia Pianetti, Marina Noris, Timothy H. J. Goodship, and John P. Atkinson Blood Volume 111(2):624-632 January 15, 2008 ©2008 by American Society of Hematology MCP structure. Celia J. Fang et al. Blood 2008;111:624-632 ©2008 by American Society of Hematology Expression profile and electrophoretic mobility of R69W and A304V mutants. Celia J. Fang et al. Blood 2008;111:624-632 ©2008 by American Society of Hematology Analysis of MCP binding to C3b and C4b. Celia J. Fang et al. Blood 2008;111:624-632 ©2008 by American Society of Hematology Analysis of C3b and C4b cofactor activity. Celia J. Fang et al. Blood 2008;111:624-632 ©2008 by American Society of Hematology Comparison of R69W mutant versus wild-type MCP in inhibiting the alternative complement pathway. Celia J. Fang et al. Blood 2008;111:624-632 ©2008 by American Society of Hematology Comparison of A304V mutant versus wild-type MCP in inhibiting the alternative pathway. Celia J. Fang et al. Blood 2008;111:624-632 ©2008 by American Society of Hematology Kinetic analysis of cofactor activity for C4b of wild type versus the A304V mutant MCP. Control profile was nearly identical for cells exposed to sensitizing antibody but not to serum or to serum alone. Celia J. Fang et al. Blood 2008;111:624-632 ©2008 by American Society of Hematology
