title: Diagnosis of Pompe's disease using pyridylamino-maltooligosaccharides as substrates of alpha-1,4-glucosidase.
abstract: We have developed a sensitive method for the assay of alpha-1,4-glucosidase in cultured skin fibroblasts and muscle tissue using pyridylamino-maltooligosaccharides as fluorescent substrates. This method is useful for the diagnosis of Pompe's disease.
mesh_terms: D000631:Aminopyridines / Q000378:metabolism*; D002478:Cells, Cultured; D002851:Chromatography, High Pressure Liquid; D005347:Fibroblasts / Q000201:enzymology; D005959:Glucosidases / Q000032:analysis*; D006008:Glycogen Storage Disease / Q000175:diagnosis*; D006009:Glycogen Storage Disease Type II / Q000175:diagnosis* / Q000201:enzymology; D006801:Humans; D006863:Hydrogen-Ion Concentration; D008214:Lymphocytes / Q000201:enzymology; D009132:Muscles / Q000201:enzymology; D009844:Oligosaccharides / Q000378:metabolism*; D000520:alpha-Glucosidases / Q000032:analysis* / Q000378:metabolism
chemical_list: D000631:Aminopyridines; D009844:Oligosaccharides; D005959:Glucosidases; D000520:alpha-Glucosidases