Presence of activator proteins for the enzymatic degradation of glucosylceramide in several human tissues.
Glucosylceramidase (EC 3.2.1.45) protein activators, similar to the 'placental factor' previously identified by us in human placenta, have also been found in human liver, normal and Gaucher fibroblasts and Gaucher spleen. They stimulate enzymatic hydrolysis of the natural substrate, glucosylceramide, but not that of the artificial substrate, 4-MU-beta-D-glucopyranoside. They are present in the tissues over the minimum amount necessary for full activation of the enzyme and must be eliminated from crude enzyme preparations in order to observer their effect on glucosylceramidase activity. The factors are not tissue-specific in that the factors from any one of the sources can activate glucosylceramidase from either placenta or liver. The presence of taurocholate or phosphatidylserine in the assay is essential for the factor efficiency. A normal level of the activator proteins was found in fibroblasts from subjects affected with Gaucher disease type I, type II and type III.
Vaccaro
A M
AM
Department of Metabolism and Pathological Biochemistry, Istituto Superiore di Sanità, Rome, Italy.
Ciaffoni
F
F
Mandara
I
I
Suzuki
K
K
D002478:Cells, Cultured; D004789:Enzyme Activation; D005260:Female; D005347:Fibroblasts / Q000201:enzymology; D005776:Gaucher Disease / Q000201:enzymology; D005959:Glucosidases / Q000378:metabolism*; D005962:Glucosylceramidase / Q000378:metabolism*; D005963:Glucosylceramides / Q000378:metabolism; D006801:Humans; D008099:Liver / Q000201:enzymology; D010920:Placenta / Q000201:enzymology; D011247:Pregnancy; D013154:Spleen / Q000201:enzymology
D005963:Glucosylceramides; D005959:Glucosidases; D005962:Glucosylceramidase