Oral glutamine and amino acid supplementation inhibit whole-body protein degradation in children with Duchenne muscular dystrophy
Oral glutamine and amino acid supplementation inhibit whole-body protein degradation in children with Duchenne muscular dystrophy
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DOI：
10.1093/ajcn/83.4.823
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发表时间：
2006-04-01
影响因子：
7.1
通讯作者：
Hankard, R
中科院分区：
文献类型：
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作者：
Mok, E;Eléouet-Da Violante, C;Hankard, R
背景：谷氨酰胺已被证明能急剧降低杜氏肌营养不良（DMD）患者的全身蛋白质降解。目的：为了改善DMD患者的营养支持，我们测试了口服谷氨酰胺10 d是否比异氮氨基酸对照混合物更能显著降低全身蛋白质降解。设计：26名患有DMD的男孩被纳入这项随机、双盲平行研究；他们口服谷氨酰胺(0.5 g(.) kg（-1) d(-1)）或等氮非特异性氨基酸混合物(0.8 (.)g kg(-1) - d(-1)) 10天。各组受试者在进入时没有临床差异。通过在补充前和补充后10 d连续静脉输注[1-C-13]亮氨酸和[2-N-15]谷氨酰胺来评估吸收后状态下亮氨酸和谷氨酰胺的代谢。结果：观察到时间对全身蛋白质降解的估计有显著影响。在补充谷氨酰胺和异氮氨基酸后，发现亮氨酸出现率（全身蛋白质降解指数）显著（P < 0.05）降低[(x) over bar +/- SEM：谷氨酰胺为136 +/- 9至124 +/- 6 μ mol / kg无脂质量（FFM）（-1） （.） h(-1)，氨基酸为136 +/- 6至131 +/- 8 μ mol (.) kg FFM-1 (.) h(-1)]。蛋白质分解导致内源谷氨酰胺含量显著（P < 0.05）降低（91 +/- 6 ~ 83 +/- 4 μ mol (.) kg FFM-1）。h（-1）为谷氨酰胺，91 +/- 4 ~ 88 +/- 5 μ mol (.) kg FFM-1 （.) h（-1）为氨基酸）。在两个补充组之间，全身蛋白质降解估计值的降低没有显著差异。结论：口服谷氨酰胺或氨基酸超过10 d均可抑制DMD全身蛋白质降解。
Background: Glutamine has been shown to acutely decrease wholebody protein degradation in Duchenne muscular dystrophy (DMD).Objective: To improve nutritional support in DMD, we tested whether oral supplementation with glutamine for 10 d decreased whole-body protein degradation significantly more than did an iso-nitrogenous amino acid control mixture.Design: Twenty-six boys with DMD were included in this randomized, double-blind parallel study; they received an oral supplement of either glutamine (0.5 g (.) kg(-1) d(-1)) or an isonitrogenous, nonspecific amino acid mixture (0.8 (.) g kg(-1) - d(-1)) for 10 d. The subjects in each group were not clinically different at entry. Leucine and glutamine metabolisms were estimated in the postabsorptive state by using a primed continuous intravenous infusion of [1-C-13] leucine and [2-N-15]glutamine before and 10 d after supplementation.Results: A significant effect of time was observed on estimates of whole-body protein degradation. A significant (P < 0.05) decrease in the rate of leucine appearance (an index of whole-body protein degradation) was observed after both glutamine and isonitrogenous amino acid supplementation [(x) over bar +/- SEM: 136 +/- 9 to 124 +/- 6 mu mol kg fat-free mass (FFM)(-1) (.) h(-1) for glutamine and 136 +/- 6 to 131 +/- 8 mu mol (.) kg FFM-1 (.) h(-1) for amino acids]. A significant (P < 0.05) decrease in endogenous glutamine due to protein breakdown was also observed (91 +/- 6 to 83 +/- 4 mu mol (.) kg FFM-1 . h(-1) for glutamine and 91 +/- 4 to 88 +/- 5 mu mol (.) kg FFM-1 (.) h(-1) for amino acids). The decrease in the estimates of whole-body protein degradation did not differ significantly between the 2 supplemental groups.Conclusion: Oral glulamine or amino acid supplementation over 10 d equally inhibits whole-body protein degradation in DMD.