基因名:
ACADM
产品别名:
ACAD1; MCAD; MCADH; ACADM; acyl-CoA dehydrogenase medium chain; acyl-CoA dehydrogenase medium chain; medium-chain specific acyl-CoA dehydrogenase, mitochondrial; acyl-CoA dehydrogenase, C-4 to C-12 straight chain; acyl-Coenzyme A dehydrogenase, C-4 to C-12 straight chain; medium-chain acyl-CoA dehydrogenase; testicular tissue protein Li 7; 酰基辅酶A脱氢酶C4-C12直链(ACADM); 酰基辅酶A脱氢酶中链;
背景信息:
Medium-Chain Specific Acyl-CoA Dehydrogenase (ACADM) is a mitochondrial fatty acid beta-oxidation that belongs to the acyl-CoA dehydrogenase family. ACADM is a homotetramer enzyme that catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. ACADM is specific for acyl chain lengths of 4 to 16. It is essential for converting these particular fatty acids to energy, especially during fasting periods. Defects in ACADM cause medium-chain acyl-CoA dehydrogenase deficiency, a disease characterized by hepatic dysfunction, fasting hypoglycemia, and encephalopathy, which can result in infantile death.