Enzyme deficient in maple syrup urine disease:
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 sugar/maple syrup smelling urine.
Branched chain alpha keto acid decarboxylase
Methionine adenosyl transferase
Fumarylacetoacetate hydrolase
Tyrosine aminotransferase
Proteins and Amino Acids