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Consequences of excessive glucosylsphingosine in glucocerebrosidase-deficient zebrafish.

Abstract: 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 s...

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Principais autores: Lindsey T. Lelieveld, Sophie Gerhardt, Saskia Maas, Kimberley C. Zwiers, Claire de Wit, Ernst H. Beijk, Maria J. Ferraz, Marta Artola, Annemarie H. Meijer, Christian Tudorache, Daniela Salvatori, Rolf G. Boot, Johannes M.F.G. Aerts
Formato: Artigo
Idioma:Inglês
Publicado em: Elsevier 2022-05-01
coleção:Journal of Lipid Research
Assuntos:
Gaucher disease
acid ceramidase
lipid metabolism
zebrafish
glucosylceramide
sphingolipids
Acesso em linha:http://www.sciencedirect.com/science/article/pii/S0022227522000323
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