Intracellular degradation of fluorescent glycolipids by lysosomal enzymes and their activators.

Abstract excerpt

Fluorescent glycolipids were utilized for detection of the intracellular, activator-dependent, activities of beta-glucocerebrosidase and arylsulphatase A. Activities were measured in primary skin fibroblasts from normal individuals, from patients with Gaucher disease who had mutations within the beta-glucocerebrosidase gene, and from a prosaposin-deficient patient. Fluorescent microscopy demonstrated that...

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