Maple syrup urine disease, one of the inherited metabolic disorder is caused because of blockage of oxidative decarboxylation of α keto acids derived from valine, leucine and isoleucine. Which of the following enzyme is missing or defective in the patient ?
1
Homogentisate reductase
2
Branched chain dehydrogenase
3
Oxaloacetate decarboxylase
4
Acetoacetate carboxylase