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. 2000 Sep 12;55(5):710-2.
doi: 10.1212/wnl.55.5.710.

The effects of l-alanine supplementation in late-onset glycogen storage disease type II

O A Bodamer  1 , D Halliday, J V Leonard
Affiliations

The effects of l-alanine supplementation in late-onset glycogen storage disease type II

O A Bodamer et al. Neurology. .

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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