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What is An inherited deficiency of branched chain a keto acid dehydrogenase result in which condition? and explain in detail?
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.