The effects of l-alanine supplementation in late-onset glycogen storage disease type II
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Published on: December 23, 2014
O A Bodamer1, D Halliday, J V Leonard
1Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA. obodamer@bcm.tmc.edu
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.
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