An inherited deficiency of branched chain a keto acid dehydrogenase result in which condition?

Maple syrup urine disease is caused by a deficiency of branched-chain a-keto acid dehydrogenase. This enzyme is involved in the catabolism of the branched- chain amino acids leucine, isoleucine, and valine. The sweet-smelling intermediates that accumulate in the urine give the disease its name. Neurologic problems are common in patients with this disease. Phenylketonuria is caused by a deficiency of phenylalanine hydroxylase. Homocystinuria is caused by a deficiency of cystathionine synthetase. A deficiency of homogentisate oxidase results in alcaptonuria, and a lack of histidase causes histidinemia.