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 journalArticle

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

Fingerprint

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

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

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 journalArticle

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.
@article{7bfd28fe194c4c1ba1ce44a28170de5c,
title = "The effects of L-alanine supplementation in late-onset glycogen storage disease type II",
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.",
author = "Bodamer, {O. A F} and D. Halliday and Leonard, {J. V.}",
year = "2000",
month = "9",
day = "20",
language = "English",
volume = "55",
pages = "710--712",
journal = "Neurology",
issn = "0028-3878",
publisher = "Lippincott Williams and Wilkins",
number = "5",

}

TY - JOUR

T1 - The effects of L-alanine supplementation in late-onset glycogen storage disease type II

AU - Bodamer, O. A F

AU - Halliday, D.

AU - Leonard, J. V.

PY - 2000/9/20

Y1 - 2000/9/20

N2 - 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.

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.

UR - http://www.scopus.com/inward/record.url?scp=0033811154&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0033811154&partnerID=8YFLogxK

M3 - Article

VL - 55

SP - 710

EP - 712

JO - Neurology

JF - Neurology

SN - 0028-3878

IS - 5

ER -