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Proteomic analysis unveils Gb3-independent alterations and mitochondrial dysfunction in a gla(−/−) zebrafish model of Fabry disease
BACKGROUND: Fabry disease (FD) is a rare lysosomal storage disorder caused by mutations in the GLA gene, resulting in reduced or lack of α-galactosidase A activity. This results in the accumulation of globotriaosylceramide (Gb3) and other glycosphingolipids in lysosomes causing cellular impairment a...
Autores principales: | Elsaid, Hassan Osman Alhassan, Rivedal, Mariell, Skandalou, Eleni, Svarstad, Einar, Tøndel, Camilla, Birkeland, Even, Eikrem, Øystein, Babickova, Janka, Marti, Hans-Peter, Furriol, Jessica |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2023
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10478213/ https://www.ncbi.nlm.nih.gov/pubmed/37670295 http://dx.doi.org/10.1186/s12967-023-04475-y |
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