Biochemistry Enzyme defective in Maple syrup urine disease is: A Tyrosinase B Phenylalanine hydroxylase C Branched chain alpha ketoacid dehydrogenase D Homogentisate oxidase 💡 Explanation Enzyme defective in Maple syrup urine disease is: ✓ Correct Answer: C. Branched chain alpha ketoacid dehydrogenase