<prompt> Branched-chain ketoaciduria is due to deficiency ofCarboxylasea-ketoacid dehydrogenaseMethyl-malonyl CoA mutaseFumaryacetoacetate hydrolase  <response>: Maple syrup urine disease MSUD, also called branched-chain ketoaciduria, is an autosomal recessive metabolic disorder affecting branched-chain amino acids. It is one type of organic acidemia. The condition gets its name from the distinctive sweet odor of affected infants; urine, paicularly prior to diagnosis, and during times of acute illness MSUD is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex BCKDC, leading to a buildup of the branched-chain amino acids leucine, isoleucine, and valine and their toxic by-products ketoacids in the blood and urine. 