Kelts D G, Ney D, Bay C, Saudubray J M, Nyhan W L
Pediatr Res. 1985 Jan;19(1):86-91. doi: 10.1203/00006450-198501000-00023.
Two infants with disorders of propionate metabolism were studied at 7 months of age to determine optimum levels of intake of protein and calories to meet the requirements for essential amino acid for growth in infancy, and at the same time minimize the accumulation of toxic intermediates. An effect of alanine was found that permitted growth at otherwise limiting levels of protein intake. This was not simply an effect of nonessential nitrogen as neither glycine nor glutamic acid could substitute for alanine in this protein-sparing effect. This appears to represent further evidence of the relationship between alanine and the branched-chain amino acids and of the importance of the alanine-glucose cycle in human physiology.
对两名患有丙酸代谢紊乱的婴儿在7个月大时进行了研究，以确定蛋白质和热量的最佳摄入量，以满足婴儿生长对必需氨基酸的需求，同时尽量减少有毒中间产物的积累。发现丙氨酸具有一种作用，能使婴儿在原本限制蛋白质摄入的水平下实现生长。这并非仅仅是非必需氮的作用，因为在这种节省蛋白质的作用中，甘氨酸和谷氨酸都无法替代丙氨酸。这似乎进一步证明了丙氨酸与支链氨基酸之间的关系，以及丙氨酸 - 葡萄糖循环在人体生理学中的重要性。
