Developmental study of α-glucosidases in japanese quails with acid maltase deficiency

✍ Scribed by Fusako Usuki; Shoichi Ishiura; Dr. Hideo Sugita

• Publisher: John Wiley and Sons
• Year: 1986
• Tongue: English
• Weight: 516 KB
• Volume: 9
• Category: Article
• ISSN: 0148-639X
• DOI: 10.1002/mus.880090610

✦ Synopsis

In Japanese quails with late-onset acid maltase deficiency (AMD), the activity of acid a-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 a-glucosidase. However, in affected embryos, the glycogen content and other lysosomal enzyme activities were normal, despite the low acid a-glucosidase activity. In a separate study, we found the existence of two age-dependent neutral a- glucosidases-"embryonic"

and "adult" a-glucosidases. In affected quails, the transition from the embryonic neutral a-glucosidase to the adult type was not influenced by the disease. The activity toward maltose and glycogen of the embryonic neutral a-glucosidase may explain the normal glycogen content in the affected embryos.