In the setting of methylmalonic acidemia, methylmalonyl-CoA accretion may increase the activity of the branched-chain alpha-ketoacid dehydrogenase complex by inhibiting the branched-chain alpha-ketoacid dehydrogenase kinase in the skeletal muscle beds, setting up a physiological forward feeding circuit for MMA production. The gene discussed is BCKDK; the disease is methylmalonic acidemia.