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

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

doi:10.1212/WNL.55.5.710

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

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

Human Genetics

Research output: Contribution to journal › Article › peer-review

27 Scopus citations

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 language	English (US)
Pages (from-to)	710-712
Number of pages	3
Journal	Neurology
Volume	55
Issue number	5
DOIs	https://doi.org/10.1212/WNL.55.5.710
State	Published - 2000

ASJC Scopus subject areas

Clinical Neurology

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

Abstract

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