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What is Branched chain ketoaciduria is a defect of catabolism of all the following aminoacids except- and explain in detail?
Ans. is 'd' i.e., Methionine * Branched chain ketoaciduria or Maple Syrup Urine Disease (MSUD) is caused by a defect of branched chain keto acid dehydrogenase enzyme complex (BCKD). BCKD catalyses the second step of catabolism of branched chain amino acids - Leucine, Isoleucine and Valine.