Maple syrup urine disease is due to
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
a-ketoacid decarboxylase
Transaminase
Isomerase
Mutase
Metabolism of protein and amino acid