Endothelial Dysfunction in Fabry Disease Is Related to Glycocalyx Degradation
Fabry disease (FD) is an X-linked multisystemic lysosomal storage disease due to a deficiency of α-galactosidase A (GLA/AGAL). Progressive cellular accumulation of the AGAL substrate globotriaosylceramide (Gb3) leads to endothelial dysfunction. Here, we analyzed endothelial function in vivo and in v...
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Autores principales: | , , , , |
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Formato: | article |
Lenguaje: | EN |
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Frontiers Media S.A.
2021
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Acceso en línea: | https://doaj.org/article/496b45111a274907bee2fcb13ab63526 |
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