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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...
Autores principales: | , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2012
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3344819/ https://www.ncbi.nlm.nih.gov/pubmed/22574107 http://dx.doi.org/10.1371/journal.pone.0033743 |