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

Abstract: 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 chole...

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Bibliografiske detaljer

Principais autores:	Daphne E. Boer, Mina Mirzaian, Maria J. Ferraz, Kimberley C. Zwiers, Merel V. Baks, Marc D. Hazeu, Roelof Ottenhoff, André R.A. Marques, Rianne Meijer, Jonathan C.P. Roos, Timothy M. Cox, Rolf G. Boot, Navraj Pannu, Herman S. Overkleeft, Marta Artola, Johannes M. Aerts
Format:	Artigo
Sprog:	Inglês
Udgivet:	Elsevier 2021-01-01
Serier:	Journal of Lipid Research
Fag:	ceramides cerebrosides Gaucher disease glycolipids inborn errors of metabolism metabolism
Online adgang:	http://www.sciencedirect.com/science/article/pii/S0022227520437411
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