Consequences of excessive glucosylsphingosine in glucocerebrosidase-deficient zebrafish.

In Gaucher disease (GD), the deficiency of glucocerebrosidase causes lysosomal accumulation of glucosylceramide (GlcCer), which is partly converted by acid ceramidase to glucosylsphingosine (GlcSph) in the lysosome. Chronically elevated blood and tissue GlcSph is thought to contribute to symptoms in...

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Detalles Bibliográficos
Autores principales: Lelieveld, Lindsey T., Gerhardt, Sophie, Maas, Saskia, Zwiers, Kimberley C., de Wit, Claire, Beijk, Ernst H., Ferraz, Maria J., Artola, Marta, Meijer, Annemarie H., Tudorache, Christian, Salvatori, Daniela, Boot, Rolf G., Aerts, Johannes M.F.G.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Biochemistry and Molecular Biology 2022
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9058576/
https://www.ncbi.nlm.nih.gov/pubmed/35315333
http://dx.doi.org/10.1016/j.jlr.2022.100199

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9058576/
https://www.ncbi.nlm.nih.gov/pubmed/35315333
http://dx.doi.org/10.1016/j.jlr.2022.100199

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