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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Bibliografske podrobnosti
izdano v:J Lipid Res
Main Authors: 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
Jezik:Inglês
Izdano: The American Society for Biochemistry and Molecular Biology 2019
Teme:
Research Articles
Online dostop: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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