The role of carnitine in normal and altered fatty acid metabolism
The role of carnitine in normal and altered fatty acid metabolism
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DOI：
10.1016/s0272-6386(03)00112-4
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发表时间：
2003-04-01
影响因子：
13.2
通讯作者：
Hoppel, C
中科院分区：
文献类型：
--
作者：
Hoppel, C
肉毒碱是一种从饲料中获得的低分子量化合物，也是由必需氨基酸赖氨酸和蛋氨酸生物合成的。肉毒碱已在多种哺乳动物组织中被发现，并通过专门的酰基转移酶的作用，在长链脂肪酸的线粒体氧化中起专性作用。肉碱的其他作用包括缓冲酰基辅酶A (CoA)-CoA比率，支链氨基酸代谢，去除多余的酰基和过氧化物酶体脂肪酸氧化。关于肉毒碱功能的证据越来越多，这使得人们对与肉毒碱代谢改变相关的疾病有了更多的了解和认识。脂肪酸氧化和代谢紊乱通常与原发性和继发性肉碱缺乏症有关。这些疾病，包括脂肪分解增加、脂质过氧化增加、酰基肉碱积累和膜通透性改变，对心肌疾病和肾衰竭患者有显著后果。治疗性给予肉毒碱在治疗改变肉毒碱内稳态的特定患者组中显示出希望，这些患者改善了心功能，增加了运动能力，减少了肌肉痉挛，减少了分析并发症。
Carnitine is a low-molecular-weight compound obtained from the diet that also is biosynthesized from the essential amino acids lysine and methionine. Carnitine has been identified in a variety of mammalian tissues and has an obligate role in the mitochondrial oxidation of long-chain fatty acids through the action of specialized acyltransferases. Other roles for carnitine include buffering of the acyl coenzyme A (CoA)-CoA ratio, branched-chain amino acid metabolism, removal of excess acyl groups, and peroxisomal fatty acid oxidation. The growing body of evidence about carnitine function has led to increased understanding and identification of disorders associated with altered carnitine metabolism. Disorders of fatty acid oxidation and metabolism typically are associated with primary and secondary forms of carnitine deficiency. These disorders, which include increased lipolysis, increased lipid peroxidation, accumulation of acylcarnitines, and altered membrane permeability, have significant consequences for patients with myocardial diseases and kidney failure. Therapeutic administration of carnitine shows promise in treating selected groups of patients who have altered carnitine homeostasis, resulting in improved cardiac function, increased exercise capacity, reduced muscle cramps, and reduced intradialytic complications.