title: Glycosidases in human skin fibroblast cultures. Alpha-fucosidase, alpha-galactosidase, alpha-glucosidase, beta-mannosidase, and N-acetyl-alpha-glucosaminidase.
abstract: Five glycosidases, alpha-fucosidase, alpha-galactosidase, alpha-glucosidase, beta-mannosidase and N-acetyl-alpha-glucosaminidase were studied in human skin fibroblast cultures. The pH-dependency, kinetic properties of the enzymes and results of isoelectric focusing and ion exchange chromatography are presented. Techniques suitable for diagnosing inborn lysosomal diseases in skin fibroblast cultures are defined.
mesh_terms: D000293:Adolescent; D009202:Cardiomyopathies / Q000201:enzymology; D002478:Cells, Cultured; D002648:Child; D002852:Chromatography, Ion Exchange; D003550:Cystic Fibrosis / Q000201:enzymology; D005260:Female; D005347:Fibroblasts / Q000201:enzymology*; D005696:Galactosidases / Q000032:analysis; D005959:Glucosidases / Q000032:analysis / Q000378:metabolism*; D006008:Glycogen Storage Disease / Q000201:enzymology; D006026:Glycoside Hydrolases / Q000032:analysis; D006596:Hexosaminidases / Q000032:analysis; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007525:Isoelectric Focusing; D008297:Male; D009083:Mucopolysaccharidoses / Q000201:enzymology; D012867:Skin / Q000166:cytology*; D013577:Syndrome
chemical_list: D005696:Galactosidases; D005959:Glucosidases; D006026:Glycoside Hydrolases; D006596:Hexosaminidases