Collins J E, Umpleby A M, Boroujerdi M A, Leonard J V, Sonksen P H
Pediatr Res. 1987 Jan;21(1):10-3. doi: 10.1203/00006450-198701000-00004.
Leucine turnover was measured using [1-14C] L-leucine in three patients with classical maple syrup urine disease. There was measurable leucine oxidation although it was lower than in normal adults. Leucine production rate was greater than normal in all three patients with an increased rate of incorporation of leucine into protein and increased protein catabolism. These fluxes were both relatively insensitive to exogenous insulin.
使用[1-14C]L-亮氨酸对三名经典型枫糖尿症患者的亮氨酸周转率进行了测量。尽管亮氨酸氧化低于正常成年人，但仍可测量到。所有三名患者的亮氨酸生成率均高于正常水平，亮氨酸掺入蛋白质的速率增加，蛋白质分解代谢增加。这些通量对外源性胰岛素均相对不敏感。
