Maple syrup urine disease is due accumulation of -

Ans. is -c' i.e., Branched chain amino acids Maple syrup urine disease (MSUD) or branched chain ketoaciduriao It is an inborn error of metabolism of branched chain amino acids valine, leucine and isoleucine.o It is due to deficiency of enzyme that catalyzes the second reaction in these amino acids metabolism i.e. branched chain-a keto acid dehydrogenase which catalyses decarboxylationQof branched chain amino acidsQ.o As a result, the branched chain amino acids, leucineQ, isoleucineQ and valineQ, and their ct-keto acids accumulate in blood, urine and CSF.o There is characteristic maple syrup odor to the urine, o Maple syrup urine disease is due to deficiency of a-ketoacid dehydrogenase (also called a-ketoacid decarboxylase).Maple syrup urine disease (MSUD) or branched chain ketoaciduriao It is an inborn error of metabolism of branched chain amino acids valine, leucine and isoleucine,o It is due to deficiency of enzyme that catalyzes the second reaction in these amino acids metabolism i.e. branched chain-a keto acid dehydrogenase which catalyses decarboxylationQ of branched chain amino acidsQ,o As a result, the branched chain amino acids, leucineQ, isoleucineQ and valineQ and their a-keto acids accumulate in blood, urine and CSF.o There is characteristic maple svrup odor to the urine.