Cardiomyopathy and response to enzyme replacement therapy in a male mouse model for Fabry disease.

Fabry disease is an X-linked disorder of glycosphingolipid metabolism that results in progressive accumulation of neutral glycosphingolipids, (predominately globotriaosylceramide; GL-3) in lysosomes, as well as other cellular compartments and the extracellular space. Our aim was to characterize the...

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Auteurs principaux: Aurelie Nguyen Dinh Cat, Brigitte Escoubet, Vincent Agrapart, Violaine Griol-Charhbili, Trenton Schoeb, Wenguang Feng, Edgar Jaimes, David G Warnock, Frederic Jaisser
Format: article
Langue:EN
Publié: Public Library of Science (PLoS) 2012
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R
Q
Accès en ligne:https://doaj.org/article/bea0ae7dd6704052befae85e8921d938
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