MDCAT Biology MCQ #3313

In a patient with maple syrup urine disease, which enzyme's deficiency leads to the accumulation of branched-chain amino acids?

MDCAT Biology MCQ #3313

  1. Question 1

    Q1. In a patient with maple syrup urine disease, which enzyme's deficiency leads to the accumulation of branched-chain amino acids?

    • A) Branched-chain alpha-keto acid dehydrogenase
    • B) Branched-chain aminotransferase
    • C) Isovaleryl-CoA dehydrogenase
    • D) Methylmalonyl-CoA mutase

    Answer: Branched-chain alpha-keto acid dehydrogenase

    Explanation: Branched-chain alpha-keto acid dehydrogenase deficiency causes maple syrup urine disease