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A new group of glucocerebrosidase isozymes found in human white blood cells

Authors
Publisher
Elsevier Inc.
Publication Date
Volume
97
Issue
3
Identifiers
DOI: 10.1016/0006-291x(80)91489-8
Disciplines
  • Medicine

Abstract

Abstract Multiple isozymes of glucocerebroside-β-glucosidase (EC 3.2.1.45) from normal white blood cell preparations have been demonstrated utilizing flat-bed isoelectric focusing on a granulated gel. Normal human leukocyte preparations contain significant glucocerebrosidase isozyme activities with pI's between 3.2 and 3.9. These novel, acidic pI species have an average D  [1- 14C] glucocerebroside to 4-methylumbelliferyl-β-D-glucopyranoside substrate ratio greater than 40:1. β- D  -Glucocerebrosidase activity of these low pI species is markedly reduced or absent in white blood cell preparations from patients with Type I Gaucher's disease.

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