Zhu Jin-ming, Yang Zi
Department of Gynecology and Obstetrics, Peking University Third Hospital, Beijing 100083, China.
Beijing Da Xue Xue Bao Yi Xue Ban. 2006 Apr 18;38(2):214-7.
Mitochondrial fatty acids beta-oxidation is a repetitive process of four steps which provides the major source of energy for heart, liver and skeletal muscle. Several enzymes are involved in this spiral cycle. The medium-chain acyl-CoA dehydrogenase (MCAD), the short-chain acyl-CoA dehydrogenase (SCAD), the long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD) and the carnitine-palmitoyl-CoA transferase II (CPT II) deficiency have been recognized as the most common inborn errors of metabolism and frequently reported in their association with sudden infant death (SID). The prevalent mutations in these genes need further investigation in different populations.
线粒体脂肪酸β氧化是一个由四个步骤组成的重复过程，为心脏、肝脏和骨骼肌提供主要能量来源。几种酶参与了这个循环过程。中链酰基辅酶A脱氢酶（MCAD）、短链酰基辅酶A脱氢酶（SCAD）、长链3-羟基酰基辅酶A脱氢酶（LCHAD）和肉碱-棕榈酰辅酶A转移酶II（CPT II）缺乏症已被认为是最常见的先天性代谢缺陷，并经常被报道与婴儿猝死（SID）有关。这些基因中的常见突变需要在不同人群中进一步研究。
