Maple syrup urine disease is due to: (A) a-ketoacid decarboxylase, (B) Transaminase, (C) Isomerase, (D) Mutase
Answer is A. Maple syrup urine disease is also known as Branched chain ketonuria. The basic biochemical deformity is deficient decarboxylation of branched-chain keto acid. This is due to the deficiency of alpha-ketoacid decarboxylase.Ref: DM Vasudevan - Textbook of Biochemistry, 7th edition, page no: 230