Developmental study of alpha-glucosidases in Japanese quails with acid maltase deficiency.
In Japanese quails with late-onset acid maltase deficiency (AMD), the activity of acid alpha-glucosidase was severely reduced to approximately 16% of the normal level from an embryonic age. The kinetic characteristics and inhibition by Zn indicated that the residual activity was responsible for the intrinsic activity of acid alpha-glucosidase. However, in affected embryos, the glycogen content and other lysosomal enzyme activities were normal, despite the low acid alpha-glucosidase activity. In a separate study, we found the existence of two age-dependent neutral alpha-glucosidases--"embryonic" and "adult" alpha-glucosidases. In affected quails, the transition from the embryonic neutral alpha-glucosidase to the adult type was not influenced by the disease. The activity toward maltose and glycogen of the embryonic neutral alpha-glucosidase may explain the normal glycogen content in the affected embryos.
D000818:Animals; D003370:Coturnix; D004625:Embryo, Nonmammalian; D005087:Glucan 1,4-alpha-Glucosidase / Q000172:deficiency* / Q000378:metabolism; D005959:Glucosidases / Q000172:deficiency* / Q000378:metabolism*; D006003:Glycogen / Q000378:metabolism; D065089:Glycoside Hydrolase Inhibitors; D006863:Hydrogen-Ion Concentration; D007527:Isoenzymes / Q000378:metabolism; D007700:Kinetics; D009132:Muscles / Q000201:enzymology*; D009468:Neuromuscular Diseases / Q000201:enzymology* / Q000235:genetics; D010369:Pectoralis Muscles / Q000201:enzymology; D015032:Zinc / Q000494:pharmacology; D000520:alpha-Glucosidases / Q000378:metabolism*
D065089:Glycoside Hydrolase Inhibitors; D007527:Isoenzymes; D006003:Glycogen; D005959:Glucosidases; D000520:alpha-Glucosidases; D005087:Glucan 1,4-alpha-Glucosidase; D015032:Zinc