Consequences of excessive glucosylsphingosine in glucocerebrosidase-deficient zebrafish

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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Bibliographic Details
Main Authors: Aerts, J.M.F.G (Author), Artola, M. (Author), Beijk, E.H (Author), Boot, R.G (Author), de Wit, C. (Author), Ferraz, M.J (Author), Gerhardt, S. (Author), Lelieveld, L.T (Author), Maas, S. (Author), Meijer, A.H (Author), Salvatori, D. (Author), Tudorache, C. (Author), Zwiers, K.C (Author)
Format: Article
Language:English
Published: American Society for Biochemistry and Molecular Biology Inc. 2022
Subjects:
acid ceramidase
dopaminergic neurons
glucosylceramide
lipid metabolism
lysosphingolipids
sphingolipids
Supplementary key words Gaucher disease
zebrafish
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