Mitochondria: Role of citrulline and arginine supplementation in MELAS syndrome
Mitochondria: Role of citrulline and arginine supplementation in MELAS syndrome
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DOI：
10.1016/j.biocel.2013.12.009
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发表时间：
2014-03-01
影响因子：
4
通讯作者：
Scaglia, Fernando
中科院分区：
文献类型：
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作者：
El-Hattab, Ayman W.;Emrick, Lisa T.;Scaglia, Fernando
线粒体存在于所有有核人类细胞中，并产生大部分细胞能量。线粒体疾病是由功能失调的线粒体引起的，线粒体不能产生足够的ATP来满足各种器官的能量需求。线粒体脑肌病、乳酸酸中毒和卒中样发作（MELAS）综合征是一种常见的母系遗传性线粒体疾病。有越来越多的证据表明，一氧化氮（NO）缺乏症发生在MELAS综合征，并导致血液灌注受损，显着有助于几个并发症，包括中风样发作，肌病和乳酸酸中毒。精氨酸和瓜氨酸都作为NO前体，它们的给药会导致NO产生增加，因此可能对MELAS综合征具有治疗作用。瓜氨酸比精氨酸更大程度地提高NO的产生，因此，瓜氨酸可能具有更好的治疗效果。需要进行对照研究，评估精氨酸或瓜氨酸补充剂对MELAS综合征不同临床方面的影响。(C)2014爱思唯尔有限公司版权所有。
Mitochondria are found in all nucleated human cells and generate most of the cellular energy. Mitochondrial disorders result from dysfunctional mitochondria that are unable to generate sufficient ATP to meet the energy needs of various organs. Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a frequent maternally inherited mitochondrial disorder. There is growing evidence that nitric oxide (NO) deficiency occurs in MELAS syndrome and results in impaired blood perfusion that contributes significantly to several complications including stroke-like episodes, myopathy, and lactic acidosis. Both arginine and citrulline act as NO precursors and their administration results in increased NO production and hence can potentially have therapeutic utility in MELAS syndrome. Citrulline raises NO production to a greater extent than arginine, therefore, citrulline may have a better therapeutic effect. Controlled studies assessing the effects of arginine or citrulline supplementation on different clinical aspects of MELAS syndrome are needed. (C) 2014 Elsevier Ltd. All rights reserved.