Role of Carnitine on Lipid and Carbohydrate Metagolism

为了评价肉碱对脂肪和碳水化合物代谢的影响，我们分析了先天性全身性肉碱缺乏小鼠(JVS鼠)的代谢紊乱。研究结果如下：1.在肾切片测定肉碱摄取率的实验中，纯合子突变体的钠依赖肉碱摄取率显著低于对照。这一结果表明，JVS小鼠的原发缺陷很可能与肉碱在肾脏中的重吸收系统有关。JVS小鼠表现出心肌肥大，这是由于心肌细胞增大与线粒体增大有关。肉碱可显著抑制心肌肥厚。这些结果表明肉碱缺乏在心肌肥厚的发生发展中起重要作用。

To evaluate the role of carnitine on lipid and carbohydrate metabolism, we analyzed the metabolic disorders in congenital systemic carnitine deficient mouse (jvs mouse). Results are as follows ;1. In an experiment to assay the uptake of carnitine using kidney slice, homozygous mutants showed significantly lower rates of Na-dependent carnitine uptake than controls. This results indicate that the primary defect of jvs mice is most probably related to the system for reabsorption of carnitine in the kidney.2. Jvs mice show cardiac hypertrophy which is caused by enlargement of the cardiac muscle cell associated with enlargement of mitochondria. Carnitine administration significantly suppresses the cardiac hypertrophy. These results show that carnitine deficiency plays an important role in the development of the cardiac hypertrophy.