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
[{'content': 'Branched chain ketoacid decarboxylation is defective in: \nSelect and explain. \nA. Maple syrup urine disease, B. Hartnup disease, C. Alkaptonuria, D. GMI gangliosidosis', 'role': 'user'}, {'content': 'Answer: A \nExplanation: Ans. A. Maple Syrup urine disease(Ref: Harper 30/e page 276-278)Maple Syrup Urine DiseaseBiochemical DefectDeficiency of the enzyme Branched Chain Ketoacid Dehydrogenase.Defective reaction is Defective Decarboxylation.Clinical FeaturesMental RetardationConvulsionAcidosis, ComaSmell of Burnt Sugar Tests for MSUDDiNitro Phenyl Hydrazine Test (DNPH Test)Rotheras TestEnzyme AnalysisTreatmentRestrict Branched Chain Amino AcidGive high doses Thiamine.', 'role': 'assistant'}]