Biochemical and electrochemical characterization of two variant human short-chain acyl-CoA dehydrogenases

Short-chain acyl-CoA dehydrogenase (hSCAD) catalyzes the first matrix step in the mitochondrial beta-oxidation cycle with optimal activity toward butyryl- and hexanoyl-CoA. The result provides valuable insight into the mechanistic basis for dysfunction of the common variant hSCADs and demonstrates that mutations, regardless of their position in the protein structure, have a large impact on the redox properties of the enzyme.