Essential Biochemistry - Protein Metabolism (Section 6 - No. 15)

What is the main metabolic defect causing maple syrup urine disease?

Deficiency of phenylalanine hydroxylase

Deficiency in branched-chain alpha-keto acid dehydrogenase

Deficiency of tyrosine transaminase

Disruption of the urea cycle

Maple syrup urine disease is due to a deficiency in the branched-chain alpha-keto acid dehydrogenase complex.