The effects of l -alanine supplementation in late-onset glycogen storage disease type II
- 12 September 2000
- journal article
- Published by Ovid Technologies (Wolters Kluwer Health) in Neurology
- Vol. 55 (5), 710-712
- https://doi.org/10.1212/wnl.55.5.710
Abstract
Article abstract Myopathy in glycogen storage disease type II (GSD-II) is slowly progressive. Five subjects with the late-onset form of GSD-II (age range, 15 to 47 years) and seven healthy control subjects (age range, 28 to 55 years) were studied. Following alanine supplementation, resting energy expenditure decreased in patients with GSD-II (p < 0.05) compared to values seen in control subjects. Leucine flux decreased (p < 0.004), as did leucine oxidation, to levels lower than those observed in control subjects (p < 0.001). l-Alanine reduces protein turnover and catabolism in GSD-II.Keywords
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