Carnitine deficiency disorders in children
Carnitine deficiency disorders in children
复制标题
DOI：
10.1196/annals.1320.004
复制
发表时间：
2004-01-01
期刊：
影响因子：
--
通讯作者：
Stanley, CA
中科院分区：
文献类型：
--
作者：
Stanley, CA
长链脂肪酸的线粒体氧化在长时间有氧工作期间为心脏以及骨骼肌提供重要的能量来源，并且在长期禁食期间为肝生酮提供重要的能量来源。肉毒碱穿梭负责将长链脂肪酸转移穿过线粒体内膜的屏障，以获得β-氧化的酶。穿梭由三种酶（肉毒碱棕榈酰转移酶1、肉毒碱酰基肉毒碱转位酶、肉毒碱棕榈酰转移酶2）和一种小的可溶性分子肉毒碱组成，以其长链脂肪酰基肉毒碱酯的形式运输脂肪酸。肉毒碱在饮食（动物蛋白）中提供，并且也从蛋白质催化过程中产生的三甲基赖氨酸残基以低速率合成。肉毒碱周转率（300-500 μ mol/天）为
Mitochondrial oxidation of long-chain fatty acids provides an important source of energy for the heart as well as for skeletal muscle during prolonged aerobic work and for hepatic ketogenesis during long-term fasting. The carnitine shuttle is responsible for transferring long-chain fatty acids across the barrier of the inner mitochondrial membrane to gain access to the enzymes of beta-oxidation. The shuttle consists of three enzymes (carnitine palmitoyltransferase 1, carnitine acylcarnitine translocase, carnitine palmitoyltransferase 2) and a small, soluble molecule, carnitine, to transport fatty acids as their long-chain fatty acylcarnitine esters. Carnitine is provided in the diet (animal protein) and also synthesized at low rates from trimethyl-lysine residues generated during protein catabolism. Carnitine turnover rates (300-500 mu mol/day) are