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: Solvey Pollmann, David Scharnetzki, Dominique Manikowski, Malte Lenders, Eva Brand
Formato: article
Lenguaje:EN
Publicado: Frontiers Media S.A. 2021
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Acceso en línea:https://doaj.org/article/496b45111a274907bee2fcb13ab63526
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