Branched-chain ketoaciduria is due to deficiency of
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.
Carboxylase
a-ketoacid dehydrogenase
Methyl-malonyl CoA mutase
Fumaryacetoacetate hydrolase
Metabolism of carbohydrate