title: [Study of the specificity of human lysosomal glycolipid hydrolases using synthetic fluorogenic substrates].
abstract: On the basis of o-acylamino-4-methylumbelliferon, a number of beta-galactosides and beta-glucosides have been synthesized. The fluorogenic compounds obtained differ by the length of acyl residues. 6- and 8-hexadecanoylamino-4-methylumbelliferyl-beta-D-galactopyranosides (6-HMGal and 8-HMGal) are shown to be substrates for human galactocerebroside-beta-D-galactosidase. 6-HMGal analogues with shorter acyl residues, octanoyl (OMGal) and butanoyl (BMGal), were cleaved by another type of beta-galactosidase, GM1-ganglioside-beta-galactosidase. It has been established that 6-hexadecanoylamino-4-methylumbelliferyl-beta-D-glucopyranoside (HMGlc) is cleaved by human and animal glucocerebrosidase much slower than its chromogenic analogue (HMGlc). OMGlc did not exceed HNGlc either, though it is cleaved by glucocerebrosidase faster than HMGlc.
authors.forename: G Ia
authors.initials: GIa
authors.lastname: Vidershaĭn
authors.forename: I K
authors.initials: IK
authors.lastname: Kozlova
authors.forename: G S
authors.initials: GS
authors.lastname: Il'ina
authors.forename: M A
authors.initials: MA
authors.lastname: Mikhaĭlova
mesh_terms: D002384:Catalysis; D055598:Chemical Phenomena; D002621:Chemistry; D002850:Chromatography, Gel; D004796:Clinical Enzyme Tests; D005456:Fluorescent Dyes; D005696:Galactosidases / Q000032:analysis*; D005697:Galactosides; D005698:Galactosylceramidase / Q000032:analysis* / Q000172:deficiency; D005959:Glucosidases / Q000032:analysis*; D005960:Glucosides; D005962:Glucosylceramidase / Q000032:analysis* / Q000172:deficiency; D006801:Humans; D007700:Kinetics; D008247:Lysosomes / Q000201:enzymology*; D013106:Sphingolipidoses / Q000175:diagnosis; D013379:Substrate Specificity; D001616:beta-Galactosidase / Q000032:analysis* / Q000172:deficiency
chemical_list: D005456:Fluorescent Dyes; D005697:Galactosides; D005960:Glucosides; D005696:Galactosidases; D005959:Glucosidases; D001616:beta-Galactosidase; D005962:Glucosylceramidase; D005698:Galactosylceramidase
vernacular_title: Issledovanie spetsifichnosti lizosomnykh glikolipidgidrolaz cheloveka s pomoshch'iu sinteticheskikh fluorogennykh substratov.