Abstract
Human tissues and cells contain multiple isoenzymes of β-glucosidase capable of hydrolyzing 4-methylumbelliferyl-β-glucoside (reviewed in Refs. 1, 2). Besides the lysosomal acid β-glucosidase (glucocerebrosidase, EC 3.2.1.45), a soluble, broad-specificity β-glucosidase and a tightly membrane-bound non-specific β-glucosidase have been described1. Only glucocerebrosidase is able to hydrolyze the lipid glucosylceramide (also called glucocerebroside). Deficiency of glucocerebrosidase is the metabolic basis for Gaucher disease, an inherited lysosomal storage disorder in man2. The other β-glucosidases are not deficient in Gaucher disease1.
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References
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© 1988 Plenum Press, New York
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Sa Miranda, M.C., Pinto, R., Schram, A.W., Tager, J.M., Aerts, J.M.F.G., Barranger, J.A. (1988). Heterogeneity in Human Acid β-Glucosidase with Cellulose-Acetate Electrophoresis. In: Salvayre, R., Douste-Blazy, L., Gatt, S. (eds) Lipid Storage Disorders. NATO ASI Series, vol 150. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-1029-7_9
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DOI: https://doi.org/10.1007/978-1-4613-1029-7_9
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