DBT
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). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component. Belongs to the 2-oxoacid dehydrogenase family. Note: This description may include information from UniProtKB.
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Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 2.3.1.168; Mitochondrial; Transferase |
Chromosomal Location of human Ortholog: 1p21.2 |
Cellular Component:
cytoplasm; cytosol; microtubule cytoskeleton; mitochondrial alpha-ketoglutarate dehydrogenase complex; mitochondrial matrix; mitochondrial nucleoid; mitochondrion
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Molecular Function:
acetyltransferase activity; dihydrolipoyllysine-residue (2-methylpropanoyl)transferase activity; lipoic acid binding; ubiquitin protein ligase binding
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Biological Process:
branched-chain amino acid catabolic process
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Disease: Maple Syrup Urine Disease, Type Ii
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Reference #:
P11182
(UniProtKB)
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Alt. Names/Synonyms: 52 kDa mitochondrial autoantigen of primary biliary cirrhosis; BCATE2; BCKAD E2 subunit; BCKAD-E2; BCKADE2; BCKDH-E2; BCOADC-E2; Branched chain 2-oxo-acid dehydrogenase complex component E2; branched chain acyltransferase, E2 component; Branched-chain alpha-keto acid dehydrogenase complex component E2; DBT; Dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex; Dihydrolipoamide branched chain transacylase; dihydrolipoamide branched chain transacylase E2; dihydrolipoyl transacylase; Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase; E2; E2 component of branched chain alpha-keto acid dehydrogenase complex; E2B; Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial; lipoamide acyltransferase component of mitochondrial branched-chain alpha-keto acid dehydrogenase complex; MGC9061; mitochondrial branched chain alpha-keto acid dehydrogenase transacylase subunit (E2b); ODB2
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Gene Symbols: DBT
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Molecular weight:
53,487 Da
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Basal Isoelectric point:
8.71
Predict pI for various phosphorylation states
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