title: alpha-Glucosidase isoenzymes in normal and acid maltase-deficient human skeletal muscles.
abstract: One acid alpha-glucosidase and two neutral alpha-glucosidases were separated from human skeletal muscle by DEAE-cellulose column chromatography. The appearance of the two human neutral alpha-glucosidase isoenzymes was found to be age dependent. We called them "fetal" and "adult" neutral alpha-glucosidases. The biochemical properties of the fetal and adult types of neutral alpha-glucosidases appeared to be similar to those previously reported for neutral alpha-glucosidases AB and C, respectively. The neutral alpha-glucosidase activity in the column eluate of the infantile acid maltase deficiency (AMD; 5-month-old) muscle was completely of the adult type, whereas 18% of the total neutral alpha-glucosidase activity in age-matched control muscle was of the fetal type. In contrast, the eluate of the late-onset AMD (32-year-old) muscle contained both the adult and fetal neutral alpha-glucosidases, 68 and 32%, respectively.
mesh_terms: D000328:Adult; D002848:Chromatography, DEAE-Cellulose; D005333:Fetus; D005087:Glucan 1,4-alpha-Glucosidase / Q000172:deficiency*; D005959:Glucosidases / Q000145:classification / Q000172:deficiency* / Q000378:metabolism*; D006801:Humans; D007223:Infant; D007527:Isoenzymes / Q000378:metabolism*; D008970:Molecular Weight; D009132:Muscles / Q000201:enzymology*; D012016:Reference Values; D013379:Substrate Specificity
chemical_list: D007527:Isoenzymes; D005959:Glucosidases; D005087:Glucan 1,4-alpha-Glucosidase