Branched chain ketoacid decarboxylation is defective in: (A) Maple Syrup urine disease, (B) Hartnup disease, (C) Alkaptonuria, (D) GMI Gangliosidosis
Answer is A. Maple syrup urine disease is due to deficiency of the enzyme branched chain α-keto dehydrogenase which catalyzes decarboxylation of branched - chain amino acid.