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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 (Gb(3)) leads to endothelial dysfunction. Here, we analyzed endothelial function in vivo and in...
Autores principales: | Pollmann, Solvey, Scharnetzki, David, Manikowski, Dominique, Lenders, Malte, Brand, Eva |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2021
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8670230/ https://www.ncbi.nlm.nih.gov/pubmed/34917096 http://dx.doi.org/10.3389/fimmu.2021.789142 |
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