Acyl Coenzyme A Dehydrogenase, C2-To-C3 Short Chain (ACADS)

ACADS is a tetrameric mitochondrial flavoprotein, which is a member of the acyl-CoA dehydrogenase family. This enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Mutations in this gene have been associated with Short Chain Acyl-CoA Dehydrogenase Deficiency. Like the other 4 enzymes belonging to the acyl-CoA dehydrogenase family of genes, SCAD is a tetrameric mitochondrial flavoprotein. It is synthesized as a 44-kD precursor, transported into mitochondria, and proteolytically processed to its 41-kD mature form. Comparison of SCAD and MCAD showed a high degree of homology, suggesting that these enzymes evolved from a common ancestral gene and belong to a gene family.