ACADVL Human
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Description
Product Specs
Product Science Overview
Acyl-CoA Dehydrogenase, Very Long Chain (VLCAD) is a mitochondrial enzyme that plays a crucial role in the β-oxidation of long-chain fatty acids. This enzyme is part of the acyl-CoA dehydrogenase family, which catalyzes the first step in the fatty acid β-oxidation pathway. The human recombinant form of VLCAD is produced through recombinant DNA technology, allowing for the study and therapeutic use of this enzyme.
VLCAD is a flavoprotein that requires flavin adenine dinucleotide (FAD) as a cofactor. It is encoded by the ACADVL gene located on chromosome 17p11.2–p11.13105 . The enzyme is most abundant in tissues with high energy demands, such as the heart and skeletal muscles . VLCAD catalyzes the dehydrogenation of very long-chain acyl-CoA esters, which are fatty acids with chain lengths of 14 to 20 carbon atoms .
The acyl-CoA dehydrogenase family has a dynamic evolutionary history. The family originated in the common ancestor of Archaea, Bacteria, and Eukaryota, highlighting its essential role in early metabolism . Gene duplications and lateral gene transfer events have contributed to the diversity of this enzyme family. VLCAD and its paralogs have evolved through these processes, resulting in their specific roles in mitochondrial fatty acid β-oxidation .
Mutations in the ACADVL gene can lead to VLCAD deficiency, an autosomal recessive disorder characterized by impaired mitochondrial β-oxidation of long-chain fatty acids . This condition can result in severe metabolic complications, especially during periods of increased energy demand such as fasting, physical exercise, or infections . Over 230 disease-causing mutations in the ACADVL gene have been identified, reflecting the genetic heterogeneity of VLCAD deficiency .
The human recombinant form of VLCAD is produced using recombinant DNA technology. This involves cloning the ACADVL gene into an expression vector, which is then introduced into a host cell, such as Escherichia coli or yeast. The host cells express the VLCAD protein, which can be purified and used for research or therapeutic purposes. Recombinant VLCAD is essential for studying the enzyme’s structure, function, and role in metabolic disorders.
