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Alanine Decreases the Protein Requirements of Infants with Inborn Errors of Amino Acid Metabolism

 

作者: WolffJ. A.,   KeltsD. G.,   AlgertS.,   ProdanosC.,   NyhanW. L.,  

 

期刊: Journal of Neurogenetics  (Taylor Available online 1985)
卷期: Volume 2, issue 1  

页码: 41-49

 

ISSN:0167-7063

 

年代: 1985

 

DOI:10.3109/01677068509100142

 

出版商: Taylor&Francis

 

关键词: Key words:alanine;protein requirements;growth;nitrogen balance;disorders of amino acid metabolism;alanine-glucose cycle

 

数据来源: Taylor

 

摘要:

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.

 

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