Alanine decreases the protein requirements of infants with inborn errors of amino acid metabolism.
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.
D000409:Alanine / Q000008:administration & dosage*; D000592:Amino Acid Metabolism, Inborn Errors / Q000178:diet therapy* / Q000378:metabolism; D000596:Amino Acids / Q000008:administration & dosage; D001835:Body Weight; D004044:Dietary Proteins / Q000008:administration & dosage; D005260:Female; D045782:Hemiterpenes; D006801:Humans; D007223:Infant; D008297:Male; D008764:Methylmalonic Acid / Q000097:blood; D020163:Ornithine Carbamoyltransferase Deficiency Disease; D010421:Pentanoic Acids / Q000097:blood; D011422:Propionates / Q000097:blood; D011506:Proteins / Q000378:metabolism
D000596:Amino Acids; D004044:Dietary Proteins; D045782:Hemiterpenes; D010421:Pentanoic Acids; D011422:Propionates; D011506:Proteins; C008216:isovaleric acid; D008764:Methylmalonic Acid; D000409:Alanine