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丙氨酸可降低患有氨基酸代谢先天性缺陷的婴儿的蛋白质需求量。

Alanine decreases the protein requirements of infants with inborn errors of amino acid metabolism.

作者信息

Wolff J A, Kelts D G, Algert S, Prodanos C, Nyhan W L

出版信息

J Neurogenet. 1985 Feb;2(1):41-9. doi: 10.3109/01677068509100142.

Abstract

Supplementation with alanine was found to increase growth in weight and nitrogen balance in 5 infants with a variety of inborn errors of amino acid metabolism receiving diets restricted in protein. The addition of alanine to the regimen led to a mean increase in weight of 15 g/day. This and the increased nitrogen balance of 15 mg/kg/day were highly significant statistically. In addition a dose-response effect of alanine was observed. The effect of alanine was compared with that of a supplemental mixture of essential and non-essential amino acids, lacking only those considered to be toxic in these patients. Alanine at 0.05 g/kg was as effective in promoting growth in weight as 1.05 g/kg of the amino acid mixture, while 0.25 g/kg of alanine was more effective than 0.70 g/kg of the amino acid mixture. The protein sparing anabolic effect of alanine is thought to be a reflection of the alanine glucose cycle.

摘要

研究发现,对于5名患有各种先天性氨基酸代谢紊乱且接受蛋白质限制饮食的婴儿,补充丙氨酸可增加体重增长和氮平衡。在饮食方案中添加丙氨酸使体重平均每天增加15克。这以及每天每千克体重15毫克的氮平衡增加在统计学上具有高度显著性。此外,还观察到丙氨酸的剂量反应效应。将丙氨酸的效果与一种必需和非必需氨基酸补充混合物的效果进行了比较,该混合物仅缺少那些在这些患者中被认为有毒的氨基酸。每千克体重0.05克的丙氨酸在促进体重增长方面与每千克体重1.05克的氨基酸混合物一样有效,而每千克体重0.25克的丙氨酸比每千克体重0.70克的氨基酸混合物更有效。丙氨酸的蛋白质节省合成代谢作用被认为是丙氨酸 - 葡萄糖循环的一种体现。

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