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: Biochemistry
Branched chain ketoacid decarboxylation is defective in 
Select and explain. 
A. Maple Syrup urine disease, B. Hartnup disease, C. Alkaptonuria, D. GMI Gangliosidosis
A: Maple Syrup urine disease
Answer: A: Maple Syrup urine disease 
Explanation: Maple syrup urine disease is due to deficiency of the enzyme branched chain α-keto dehydrogenase which catalyzes decarboxylation of branched - chain amino acid.