The molecular and structural bases for the association of complement C3 mutations with atypical hemolytic uremic syndrome. Issue 2 (August 2015)

• Record Type: Journal Article
• Authors: Martínez-Barricarte, Rubén; Heurich, Meike; López-Perrote, Andrés; Tortajada, Agustin; Pinto, Sheila; López-Trascasa, Margarita; Sánchez-Corral, Pilar; Morgan, B. Paul; Llorca, Oscar; Harris, Claire L.; Rodríguez de Córdoba, Santiago
• Is Part Of: Molecular immunology. Volume 66:Issue 2(2015:Aug.)
• Page Start: 263
• Page End: 273
• Publication Date: 2015-08
• Subjects: Immunochemistry -- Periodicals; Molecular biology -- Periodicals; Immunochemistry -- Periodicals; Allergy and Immunology -- Periodicals; Molecular Biology -- Periodicals; Immunochimie -- Périodiques; Biologie moléculaire -- Périodiques; Immunochemistry; Molecular biology; Periodicals; Electronic journals; 571.96
• DOI: 10.1016/j.molimm.2015.03.248
• Languages: English
• ISSNs: 0161-5890

Abstract

<abstract abstract-type="author-highlights" id="abs0005"> <title id="sect0005">Highlights</title> <sec> <p id="spar0005"> <list id="lis0005"> <list-item id="lsti0005"> <label></label> <p id="par0005">Mutations in C3 have been associated with aHUS and other glomerulopathies.</p> </list-item> <list-item id="lsti0010"> <label></label> <p id="par0010">aHUS-associated C3 mutants R592W, R161W, and I1157T impair regulation by MCP, but not by FH.</p> </list-item> <list-item id="lsti0020"> <label></label> <p id="par0190">EM analysis provides the structural basis for the functional impairment of the R161W and I1157T mutants.</p> </list-item> <list-item id="lsti0015"> <label></label> <p id="par0015">Data supports aHUS-associated C3 mutations selectively affect complement regulation on surfaces.</p> </list-item> </list> </p> </sec> </abstract>