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Acid α-glucosidase deficiency in cultured fibroblasts with phenotype 2 of acid α-glucosidase

✍ Scribed by Nicholas G. Beratis; Lorraine Wilbur; Susan L. Sklower

Book ID
115824319
Publisher
Elsevier Science
Year
1983
Tongue
English
Weight
435 KB
Volume
134
Category
Article
ISSN
0009-8981

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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 intr