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...

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Detalles Bibliográficos
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
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2023
Materias:
Research
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

Internet

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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