Characteristics of γ-Glutamyltranspeptidase in Cultured Skin Fibroblasts from Normals and Cystinotics

Abstract
The characteristics of a fluorimetrie method for assay of y-glutamyltranspeptidase (EC 2.3.2.2) in cultured human skin fibroblasts from normal donors and cystinotic patients using y-glutamyl-7-amino-4-coumarin as substrate are described. It is possible with this method to measure enzymatic activity in sonicates of cells with as little as 0.029 mmol/1 L-cystine as acceptor-substrate with only 0.20 mg of cellular protein after 30-60 min of incubation. The properties of the enzyme in cells from patients with cystine storage disease show elevated y-glutamyltranspeptidase under various incubation conditions.

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