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Mutations of Factor H Impair Regulation of Surface-bound C3b by Three Mechanisms in Atypical Hemolytic Uremic Syndrome

Atypical hemolytic uremic syndrome (aHUS) is a thrombotic microangiopathy associated with mutations in complement proteins, most frequently in the main plasma alternative pathway regulator factor H (FH). The hotspot for the FH mutations is in domains 19–20 (FH19–20) that are indispensable for FH act...

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Detalhes bibliográficos
Main Authors: Lehtinen, Markus J., Rops, Angelique L., Isenman, David E., van der Vlag, Johan, Jokiranta, T. Sakari
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Biochemistry and Molecular Biology 2009
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2708861/
https://ncbi.nlm.nih.gov/pubmed/19351878
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M900814200
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