Human glucocerebrosidase mediates formation of xylosyl-cholesterol by β-xylosidase and transxylosidase reactions

Deficiency of glucocerebrosidase (GBA), a lysosomal β-glucosidase, causes Gaucher disease. The enzyme hydrolyzes β-glucosidic substrates and transglucosylates cholesterol to cholesterol-β-glucoside. Here we show that recombinant human GBA also cleaves β-xylosides and transxylosylates cholesterol. Th...

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Detalhes bibliográficos
Publicado no:J Lipid Res
Main Authors: Boer, Daphne E., Mirzaian, Mina, Ferraz, Maria J., Zwiers, Kimberley C., Baks, Merel V., Hazeu, Marc D., Ottenhoff, Roelof, Marques, André R.A., Meijer, Rianne, Roos, Jonathan C.P., Cox, Timothy M., Boot, Rolf G., Pannu, Navraj, Overkleeft, Herman S., Artola, Marta, Aerts, Johannes M.
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Biochemistry and Molecular Biology 2021
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7903134/
https://ncbi.nlm.nih.gov/pubmed/33361282
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1194/jlr.RA120001043
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