<prompt> Enzyme deficient in maple syrup urine disease:Branched chain alpha keto acid decarboxylaseMethionine adenosyl transferaseFumarylacetoacetate hydrolaseTyrosine aminotransferase  <response>: Ans. A Branched chain alpha keto acid decarboxylase Maple syrup urine disease is an inherited disorder characterized by the deficiency of branched-chain alpha- keto acid decarboxylase complex. This enzyme is required for breakdown of specific amino acid in body like valine, isoleucine and leucine. Due to the enzyme deficiency, these amino acid is not metabolized and hence there is accumulation in cells and body fluids - producing burnt sugarmaple syrup smelling urine. 