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

O. A F Bodamer, D. Halliday, J. V. Leonard

Research output: Contribution to journal › Article

25 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)710-712
Number of pages3
JournalNeurology
Volume55
Issue number5
StatePublished - Sep 20 2000

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Glycogen Storage Disease Type II
Alanine
Leucine
Muscular Diseases
Energy Metabolism
Healthy Volunteers
Proteins

ASJC Scopus subject areas

  • Neuroscience(all)

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Bodamer, O. A. F., Halliday, D., & Leonard, J. V. (2000). The effects of L-alanine supplementation in late-onset glycogen storage disease type II. Neurology, 55(5), 710-712.

The effects of L-alanine supplementation in late-onset glycogen storage disease type II. / Bodamer, O. A F; Halliday, D.; Leonard, J. V.

In: Neurology, Vol. 55, No. 5, 20.09.2000, p. 710-712.

Research output: Contribution to journal › Article

Bodamer, OAF, Halliday, D & Leonard, JV 2000, 'The effects of L-alanine supplementation in late-onset glycogen storage disease type II', Neurology, vol. 55, no. 5, pp. 710-712.
Bodamer OAF, Halliday D, Leonard JV. The effects of L-alanine supplementation in late-onset glycogen storage disease type II. Neurology. 2000 Sep 20;55(5):710-712.
Bodamer, O. A F ; Halliday, D. ; Leonard, J. V. / The effects of L-alanine supplementation in late-onset glycogen storage disease type II. In: Neurology. 2000 ; Vol. 55, No. 5. pp. 710-712.
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