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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...
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. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
American Society for Biochemistry and Molecular Biology
2022
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Materias: | |
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 |
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