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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Detalles Bibliográficos
Autores principales: 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: Online Artículo Texto
Lenguaje:English
Publicado: American Society for Biochemistry and Molecular Biology 2021
Materias:
Research Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7903134/
https://www.ncbi.nlm.nih.gov/pubmed/33361282
http://dx.doi.org/10.1194/jlr.RA120001043

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