This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Proteins and Amino Acids, Subject name: Biochemistry
Enzyme deficient in maple syrup urine disease: 
Select and explain. 
A. Branched chain alpha keto acid decarboxylase, B. Methionine adenosyl transferase, C. Fumarylacetoacetate hydrolase, D. Tyrosine aminotransferase
A: Branched chain alpha keto acid decarboxylase
Answer: A: Branched chain alpha keto acid decarboxylase 
Explanation: Ans. A Branched chain alpha keto acid decarboxylase* Maple syrup urine disease is an inherited disorder characterized by the deficiency of branched-chain alpha- keto acid decarboxylase complex.* This enzyme is required for breakdown of specific amino acid in body like valine, isoleucine and leucine.* Due to the enzyme deficiency, these amino acid is not metabolized and hence there is accumulation in cells and body fluids - producing burnt sugar/maple syrup smelling urine.