BCKDK and maple syrup urine disease: Because deficiencies in BCAA catabolism are associated with insulin resistance, maple syrup urine disease, congenital heart disease, and heart failure,12 inhibition of BCKDK by the selective inhibitor 3,6-dichlorobenzo[b]thiophene-2-carboxylic acid (BT2) has emerged as a promising therapeutic approach for metabolic diseases and cancer in humans.44