Developmental study of alpha-glucosidases in Japanese quails with acid maltase deficiency.

Abstract

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.

• Authors: F Usuki, S Ishiura, H Sugita
• Journal: Muscle & nerve (Muscle Nerve) 1986 Jul-Aug Vol. 9 Issue 6 Pg. 537-43 ISSN: 0148-639X [Print] United States
• PMID: 3090432 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Glycoside Hydrolase Inhibitors
• Isoenzymes
• Glycogen
• Glucosidases
• alpha-Glucosidases
• Glucan 1,4-alpha-Glucosidase
• Zinc

Topics

• Animals
• Coturnix
• Embryo, Nonmammalian
• Glucan 1,4-alpha-Glucosidase (deficiency, metabolism)
• Glucosidases (deficiency, metabolism)
• Glycogen (metabolism)
• Glycoside Hydrolase Inhibitors
• Hydrogen-Ion Concentration
• Isoenzymes (metabolism)
• Kinetics
• Muscles (enzymology)
• Neuromuscular Diseases (enzymology, genetics)
• Pectoralis Muscles (enzymology)
• Zinc (pharmacology)
• alpha-Glucosidases (metabolism)