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

被引：24

作者：

Bodamer, OAF ^{[1
]}

Halliday, D

Leonard, JV

机构：

[1] Baylor Coll Med, Dept Mol & Human Genet, Houston, TX 77030 USA

[2] Inst Child Hlth, Biochem Endocrine & Metab Unit, London, England

来源：

NEUROLOGY | 2000年 / 55卷 / 05期

关键词：

D O I：

10.1212/WNL.55.5.710

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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.

引用

页码：710 / 712

页数：3

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