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首页> 外文学位 >Branched chain amino acid metabolism: Requirements in healthy adult men and patients with maple syrup urine disease.
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Branched chain amino acid metabolism: Requirements in healthy adult men and patients with maple syrup urine disease.

机译：支链氨基酸代谢：健康成年男性和枫糖浆尿病患者的需求。

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摘要

Based on evidence from a series of stable isotope studies, the dietary recommendations for branched chain amino acids (BCAA), from nitrogen balance studies, are too low. As such the dietary requirement for BCAA has been increased in the new dietary reference intake (DRI) report. To date, stable isotope studies determined the requirement of leucine and valine individually, but not isoleucine.; The requirement for total BCAA was determined by the indicator amino acid oxidation (IAAO) technique in adult men (n = 7) with an oral isotope dosing protocol in which L-[1-13C]phenylalanine was used as an indicator amino acid (IAA). To reduce the possibility of interactions among the branched chain amino acids and imbalances in the mixture affecting the estimate of requirement, we adopted the proportion of BCAA in egg protein. The rate of release of 13CO2 from the oxidation of L-[1-13C]phenylalanine (F13CO2) was measured and a two-phase linear regression crossover model applied to determine the requirement for total BCAA. The mean requirement and population safe level (upper limit of 95% Cl) of total BCAA were 144 mg·kg −1·d−1 and 210 mg·kg −1·d−1, respectively.; Once the total BCAA requirement had been determined, it was necessary to define whether any one of the BCAA was limiting. Five subjects, with known total BCAA requirement were restudied. We held constant one of the BCAA at the individual's requirement level, while the other two were reduced by 10% and 20%, in random order. Based on increases in label appearance (F 13CO2) in response to decreased intake of each of the branched chain amino acids, we concluded that valine is the first limiting branched chain amino acid in egg protein.; Maple syrup urine disease is an inherited disorder results from defects in the mitochondrial multienzyme complex branched chain ketoacid dehydrogenase (BCKD; EC 1.2.4.4). Five classical MSUD patients were assigned to different intakes of total BCAA, using the indicator amino acid oxidation (IAAO) technique. Total BCAA requirement was determined by measuring the oxidation of L-[1- 13C]phenylalanine to 13CO2 (F13CO 2). The mean requirement for total BCAA was 45 mg·kg−1 ·d−1 and the safe level of intake (upper 95% confidence interval) was 62 mg·kg−1·d −1.
机译：根据一系列稳定同位素研究的证据，氮平衡研究对支链氨基酸（BCAA）的饮食建议过低。因此，在新的饮食参考摄入量（DRI）报告中增加了BCAA的饮食需求。迄今为止，稳定的同位素研究确定了亮氨酸和缬氨酸的需求量，但不是异亮氨酸的需求量。通过指示剂氨基酸氧化（IAAO）技术确定成年男性（n = 7）的口服同位素剂量方案，其中L- [1- 13 C]苯丙氨酸为用作指示氨基酸（IAA）。为了减少支链氨基酸之间的相互作用以及混合物中不平衡影响需求量估计的可能性，我们采用了鸡蛋蛋白质中BCAA的比例。 L- [1- 13 C]苯丙氨酸（F 13 13 CO 2 的速率测量super> CO 2 ），并应用两阶段线性回归交叉模型确定总BCAA的需求。 BCAA的平均需求量和人群安全水平（上限95％Cl）分别为144 mg·kg -1 ·d -1 和210 mg·kg -1 ·d -1 。一旦确定了总的BCAA要求，就必须定义是否有任何BCAA是限制性的。重新研究了五个已知总BCAA要求的受试者。在个人需求水平上，我们将BCAA中的一个保持不变，而将其他两个BCAA按随机顺序减少了10％和20％。基于响应每个支链氨基酸的摄入减少，标签外观（F 13 CO 2 ）的增加，我们得出结论缬氨酸是第一个限制性分支链卵蛋白中的氨基酸。枫糖浆尿病是一种由线粒体多酶复合物支链酮酸脱氢酶（BCKD; EC 1.2.4.4）缺陷引起的遗传性疾病。使用指示剂氨基酸氧化（IAAO）技术，将5名经典MSUD患者分配到不同的总BCAA摄入量。通过测量L- [1- 13 C]苯丙氨酸氧化为 13 CO 2 （F 13 CO 2 ）。总BCAA的平均需求量为45 mg·kg -1 ·d -1 ，安全摄入量（95％置信区间的上限）为62 mg·kg < super> -1 ·d -1 。

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  • 作者

    Riazi, Roya.;

  • 作者单位

    University of Toronto (Canada).;

  • 授予单位 University of Toronto (Canada).;
  • 学科 Health Sciences Nutrition.; Health Sciences Pathology.
  • 学位 Ph.D.
  • 年度 2003
  • 页码 225 p.
  • 总页数 225
  • 原文格式 PDF
  • 正文语种 eng
  • 中图分类 预防医学、卫生学;病理学;
  • 关键词

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