Proteintech, 85889-1-RR-50uL, ACADVL Recombinant monoclonal antibody

85889-1-RR targets ACADVL in WB, IF/ICC, ELISA applications and shows reactivity with human, mouse samples.
Background Information
ACADVL (Acyl-CoA Dehydrogenase, Very Long Chain) is a mitochondrial enzyme that catalyzes the initial step of β-oxidation for very long-chain fatty acids (VLCFAs, C14-C20). Deficiency of ACADVL causes very long-chain acyl-CoA dehydrogenase deficiency (VLCADD), the most common inherited disorder of mitochondrial fatty acid oxidation, characterized by hypoketotic hypoglycemia, cardiomyopathy, hepatopathy, and exercise-induced rhabdomyolysis. ACADVL is highly expressed in metabolically active tissues including cardiac muscle, skeletal muscle, and liver, where it plays a critical role in energy production during fasting and high-demand states. Mutations in ACADVL represent a significant diagnostic target for newborn screening and genetic counseling in metabolic disorders.