Proteomic analysis unveils Gb3-independent alterations and mitochondrial dysfunction in a gla −/− zebrafish model of Fabry disease

Proteomic analysis unveils Gb3-independent alterations and mitochondrial dysfunction in a gla −/− zebrafish model of Fabry disease

Abstract 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 impa...

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Bibliographic Details
Main Authors: Hassan Osman Alhassan Elsaid, Mariell Rivedal, Eleni Skandalou, Einar Svarstad, Camilla Tøndel, Even Birkeland, Øystein Eikrem, Janka Babickova, Hans-Peter Marti, Jessica Furriol
Format: Article
Language:English
Published: BMC 2023-09-01
Series:Journal of Translational Medicine
Subjects:
Lysosome
Mitochondria
Stress
Fabry disease
Online Access:https://doi.org/10.1186/s12967-023-04475-y

Internet

https://doi.org/10.1186/s12967-023-04475-y

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