<unk>  <unk>  is an autosomal recessive metabolic disorder of amino acid metabolism. It is also called branched chain ketoaciduria.
This amino acid acidopathy is due to a deficiency of the metabolic enzyme branched chain α-keto acid dehydrogenase (BCKDH) leading to a buildup of branched-chain amino acids (leucine, isoleucine, and valine) in the blood and<unk> .
Maple syrup urine disease
3-Methylglutaconic aciduria
Homocystinuria
Alkaptonuria
Mevalonic aciduria
Essential fructosuria
Cystinuria
Trimethylaminuria