ACADL

The protein encoded by this gene belongs to the acyl-CoA dehydrogenase family, which is a family of mitochondrial flavoenzymes involved in fatty acid and branched chain amino-acid metabolism. This protein is one of the four enzymes that catalyze the initial step of mitochondrial beta-oxidation of straight-chain fatty acid. Defects in this gene are the cause of long-chain acyl-CoA dehydrogenase (LCAD) deficiency, leading to nonketotic hypoglycemia. [provided by RefSeq, Jul 2008]该基因编码的蛋白质属于酰基辅酶A脱氢酶家族，该家族是一类参与脂肪酸和支链氨基酸代谢的线粒体黄素酶。此蛋白质是催化直链脂肪酸线粒体β氧化的初始步骤的四种酶之一。该基因的缺陷是长链酰基辅酶A脱氢酶（LCAD）缺乏症的病因，导致非酮症低血糖。[由RefSeq提供，2008年7月]