title: [A new fluorogenic substrate for human galactocerebroside-beta-D-galactosidase].
abstract: A new fluorogenic compound--6-hexadecanoylamino-4-methyl-umbelliferyl-beta-D-gala cto pyranoside (HMGal), a substrate for human galactocerebroside beta-D-galactosidase (HG), has been synthesized. A method for determining the HG activity based on the use of HMGal as a fluorogenic substrate has been developed. The specificity of HMGal hydrolysis by HG has been demonstrated in experiments with enzyme preparations from human skin fibroblasts and leukocytes in normally and in hereditary glycolipidosis (GM1-gangliosidosis and Krabbe's disease). The use of HMGal permits to markedly increase the sensitivity of the method used for determining the HG activity.
mesh_terms: D055598:Chemical Phenomena; D002621:Chemistry; D002863:Chromogenic Compounds / Q000138:chemical synthesis*; D004796:Clinical Enzyme Tests; D005677:G(M1) Ganglioside; D005696:Galactosidases / Q000032:analysis*; D005698:Galactosylceramidase / Q000032:analysis*; D005733:Gangliosidoses / Q000175:diagnosis*; D006801:Humans; D007965:Leukodystrophy, Globoid Cell / Q000175:diagnosis*
chemical_list: D002863:Chromogenic Compounds; D005677:G(M1) Ganglioside; D005696:Galactosidases; D005698:Galactosylceramidase
vernacular_title: Novyĭ fluorogenny i substrat dlia galaktotserebrozid-beta-D-galaktozidazy cheloveka.