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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Autores principales: | , , , , , , , , |
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Formato: | article |
Lenguaje: | EN |
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Public Library of Science (PLoS)
2012
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Acceso en línea: | https://doaj.org/article/bea0ae7dd6704052befae85e8921d938 |
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