| branched c. |
an open chain of atoms, usually carbon, with one or more side chains attached to it.
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| branched-chain α-keto acid dehydrogenase |
see under complex.
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| branched-chain α-keto acid dehydrogenase c. |
a multienzyme complex composed of 3-methyl-2-oxobutanoate dehydrogenase (lipoamide) [EC 1.2.4.4], dihydrolipoamide dehydrogenase [EC 1.8.1.4], and dihydrolipoamide acyltransferase, with thiamine pyrophosphate, lipoic acid, NAD+, FAD, and coenzyme A as cofactors. The integrated complex catalyzes the oxidative decarboxylation of the keto acid analogues of the branched-chain amino acids leucine, isoleucine, and valine. Deficiency of any enzyme of the complex causes maple syrup urine disease. See also lipoamide dehydrogenase deficiency.
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| branched-chain a.’s |
leucine, isoleucine, and valine; they are incorporated into proteins or catabolized for energy.
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| branched-chain k. |
any of the keto acids formed by oxidative decarboxylation of branched-chain amino acids.
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