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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Bibliographic Details
Published in: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.
Format: Artigo
Language:Inglês
Published: American Society for Biochemistry and Molecular Biology 2021
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Research Article
Online Access: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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