| 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.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| branched-chain a.’s |
leucine, isoleucine, and valine; they are incorporated into proteins or catabolized for energy.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| branched-chain k. |
any of the keto acids formed by oxidative decarboxylation of branched-chain amino acids.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| branched-chain-amino-acid transaminase |
[EC 2.6.1.42] an enzyme of the transferase class that catalyzes the removal of the α-amino group of leucine, isoleucine, or valine to form the corresponding keto acid, transferring the amino group to α-ketoglutarate or a similar acceptor. The reaction is the first step in the catabolism of branched-chain amino acids. In humans, the enzyme for leucine and isoleucine may be separate from the one for valine. Called also branched-chain-amino-acid aminotransferase.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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| brancher d. |
glycogen storage disease, type IV.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
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