左卡尼汀是将长链脂肪酸从细胞质转运至线粒体,并在此进行β-氧化的重要载体。它的缺乏是以血浆和组织中卡尼汀浓度低于组织器官发挥正常功能所需浓度为特征,包括心肌病、肌病、低酮血症、低血糖和高血氨等。研究表明,补充左卡尼汀能改善某些疾病的临床症状。 L-carnitine is an important vector for the transport of long-chain fatty acids from the cytoplasm to the mitochondria, where β-oxidation takes place. Its deficiency is characterized by a concentration of carnitine in plasma and tissue that is lower than required for normal functioning of tissues and organs, including cardiomyopathy, myopathy, hypokalemia, hypoglycemia and hypernatremia. Studies have shown that supplementation of levocarnitine can improve the clinical symptoms of certain diseases.