ACADS (acyl-Coenzyme A dehydrogenase), is a homotetrameric mitochondrial flavoprotein that belongs to the acyl-CoA dehydrogenase family. This enzyme catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. Mutation of this gene causes SCAD deficiency an acute acidosis and muscle weakness in infants and lipid-storage myopathy in adults.