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人类体内的肉碱代谢与功能

Carnitine metabolism and function in humans.

作者信息

Rebouche C J, Paulson D J

出版信息

Annu Rev Nutr. 1986;6:41-66. doi: 10.1146/annurev.nu.06.070186.000353.

Abstract

It is apparent from the foregoing discussion that carnitine plays an essential role in human intermediary metabolism. The question of a dietary requirement for carnitine, particularly for the human infant, is of significant theoretical and practical interest. Aberrant carnitine metabolism resulting from abnormal genetic or acquired conditions may have serious consequences for the affected individual. At present many of the treatment modalities for carnitine deficiency are empirical. Further clarification of the mechanisms by which carnitine depletion is manifest in these conditions is essential for designing treatment programs. Moreover, therapeutic use of carnitine in several human diseases not involving carnitine deficiency per se has been indicated. Before such treatment becomes generally accepted, we must determine precisely the role of this amino acid in the biochemical and physiological events that participate in the pathogenesis of each disease.

摘要

从上述讨论中可以明显看出,肉碱在人体中间代谢中起着至关重要的作用。关于肉碱的膳食需求问题,尤其是对人类婴儿而言,具有重大的理论和实际意义。由异常遗传或后天条件导致的肉碱代谢异常可能会给受影响的个体带来严重后果。目前,许多针对肉碱缺乏症的治疗方法都是经验性的。进一步阐明在这些情况下肉碱耗竭的表现机制对于设计治疗方案至关重要。此外,已经表明肉碱在几种本身不涉及肉碱缺乏的人类疾病中具有治疗用途。在这种治疗被普遍接受之前,我们必须准确确定这种氨基酸在参与每种疾病发病机制的生化和生理事件中的作用。

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