<prompt> A 7 day old infant presented with lethargy, decreased feeding, emesis, poor weight gain,hypotonia, a high-pitched cry, seizures, and the characteristic maple syrup smell of the urine.The following features are due to defect inOxidatonDeaminationCarboxylationDecarboxylation  <response>: Maple syrup urine disease MSUD-1 is due to impaired Alpha-Ketoacid decarboxylase component of BCKAD branched chain alpha keto acid dehydrogenase complex,leading to defective decarboxylation reaction and the formation of branched-chain amino acids leucine, isoleucine, and valine and their toxic byproducts keto-acids in blood and urine. A peculiar odor burnt sugar is found in urine, sweat, and cerumen. Treatmet:Diet lacking in branched chain amino acids Reference: Harpers illustrated biochemistry 30th edition 