... Two novel variants of human medium chain acyl-CoAdehydrogenase (MCAD)K364R, a folding mutation, and R256T, a catalytic-site mutationresulting in a well-folded but totally inactive protein Linda ... normalenzyme. Eur J Biochem 246, 548–556.15 Ikeda Y, Okamura-Ikeda K & Tanaka K (1985) Purifi-cation and characterization of short-chain, medium-chain, and long-chain acyl-CoA dehydrogenases ... I,Strauss A et al. (1997) Biochemical characterization of purified, human recombinant Lys304?Glu medium-chainacyl-CoA dehydrogenase containing the common dis-ease-causing mutation and comparison...