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肉碱缺乏及其相关疾病的研究进展
引用本文:房玥晖,蔡美琴. 肉碱缺乏及其相关疾病的研究进展[J]. 中华临床营养杂志, 2009, 17(5). DOI: 10.3760/cma.j.issn.1674-635X.2009.05.015
作者姓名:房玥晖  蔡美琴
作者单位:上海交通大学医学院营养系,200025
摘    要:肉碱是脂肪β-氧化过程中长链脂酰辅酶A透过线粒体内膜时的转运体,其缺乏导致脂质沉积性肌病、全身肉碱缺乏综合征、心肌病、肥胖、男性不育等疾病.本文主要综述肉碱缺乏的原因及所导致的相关疾病的主要临床表现和治疗手段.

关 键 词:肉碱缺乏  代谢  脂质沉积性肌病  心肌病  肥胖  男性不育

Carnitine deficiency and its related diseases
FANG Yue-hui,CAI Mei-qin. Carnitine deficiency and its related diseases[J]. Chinese Journal of Clinical Nutrition, 2009, 17(5). DOI: 10.3760/cma.j.issn.1674-635X.2009.05.015
Authors:FANG Yue-hui  CAI Mei-qin
Affiliation:FANG Yue-hui CAI Mei-qin
Abstract:L-carnitine plays an essential role in the beta-oxidation of fatty acids by transporting long chain acyl-coenzyme A into the mitochondrial matrix.Carnitine deficiency may lead to various diseases,including lipid storage myopathies,systemic carnitine deficiency syndrome,cardiomyopathy,obesity,and infertility.This article summarizes the causes of carnitine deficiency and elucidates the clinical features and treatment strategies of its related diseases.
Keywords:Carnitine deficiency  Metabolism  Lipid storage myopathies  Cardiomyopathy  Obesity  Male infertility
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