This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: None, Subject name: Unknown
Branched-chain ketoacid decarboxylation is defective in - 
Select and explain. 
A. Maple syrup urine disease, B. Hartnup disease, C. Alkaptonuria, D. GM-1 gangliosidosis
A: Maple syrup urine disease
Answer: A: Maple syrup urine disease 
Explanation: Ans-A