2coo
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Solution structure of the e3_binding domain of dihydrolipoamide branched chaintransacylase
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Structural highlights
DiseaseODB2_HUMAN Defects in DBT are the cause of maple syrup urine disease type 2 (MSUD2) [MIM:248600. MSUD is an autosomal recessive disorder characterized by mental and physical retardation, feeding problems, and a maple syrup odor to the urine.[1] [2] FunctionODB2_HUMAN The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Evolutionary Conservation![]() Check, as determined by ConSurfDB. You may read the explanation of the method and the full data available from ConSurf. References
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This page was last modified 11:27, 22 May 2024.