基因名:
ACADVL
产品别名:
ACAD6; LCACD; VLCAD; ACADVL; acyl-CoA dehydrogenase very long chain; acyl-CoA dehydrogenase very long chain; very long-chain specific acyl-CoA dehydrogenase, mitochondrial; acyl-Coenzyme A dehydrogenase, very long chain; 酰基辅酶A脱氢酶很长链; 酰基辅酶A脱氢酶短/极长链(ACADVL);
背景信息:
ACADVL (acyl-Coenzyme A dehydrogenase, very long chain) catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. It is specific to esters of long-chain and very long chain fatty acids such as palmitoyl-CoA and stearoyl-CoA. Deficiencies in ACADVL are associated with reduced myocardial fatty acid beta-oxidation and cardiomyopathy.