Role of β-glucosidase 2 in aberrant glycosphingolipid metabolism: model of glucocerebrosidase deficiency in zebrafish

β-glucosidases [GBA1 (glucocerebrosidase) and GBA2] are ubiquitous essential enzymes. Lysosomal GBA1 and cytosol-facing GBA2 degrade glucosylceramide (GlcCer); GBA1 deficiency causes Gaucher disease, a lysosomal storage disorder characterized by lysosomal accumulation of GlcCer, which is partly conv...

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Publicat a:J Lipid Res
Autors principals: Lelieveld, Lindsey T., Mirzaian, Mina, Kuo, Chi-Lin, Artola, Marta, Ferraz, Maria J., Peter, Remco E. A., Akiyama, Hisako, Greimel, Peter, van den Berg, Richard J. B. H. N., Overkleeft, Herman S., Boot, Rolf G., Meijer, Annemarie H., Aerts, Johannes M. F. G.
Format: Artigo
Idioma:Inglês
Publicat: The American Society for Biochemistry and Molecular Biology 2019
Matèries:
Research Articles
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC6824494/
https://ncbi.nlm.nih.gov/pubmed/31562193
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1194/jlr.RA119000154
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