Presku: | AAP54486 |
Size: | 100 ug |
Weight: | 64kDa |
Gene: | 37 |
Format: | Lyophilized powder |
Target: | ACADVL is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in ACADVL protein reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy.The protein encoded by this gene is targeted to the inner mitochondrial membrane where it catalyzes the first step of the mitochondrial fatty acid beta-oxidation pathway. This acyl-Coenzyme A dehydrogenase is specific to long-chain and very-long-chain fatty acids. A deficiency in this gene product reduces myocardial fatty acid beta-oxidation and is associated with cardiomyopathy. Alternative splicing results in multiple transcript variants encoding different isoforms. |
Alternative names: | ACAD6; LCACD; VLCAD |