Gene Symbol | BCKDHA |
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Entrez Gene | 282149 |
Alt Symbol | - |
Species | Bovine |
Gene Type | protein-coding |
Description | branched chain keto acid dehydrogenase E1, alpha polypeptide |
Other Description | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial|BCKDE1A|BCKDH E1-alpha|branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease)|branched-chain alpha-keto acid dehydrogenase E1 component alpha chain |
Swissprots | P11178 |
Accessions | DAA19898 P11178 BC109669 AAI09670 J03759 AAA30595 NM_174506 NP_776931 |
Function | 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). {ECO:0000269|PubMed:283398}. |
Subcellular Location | Mitochondrion matrix {ECO:0000269|PubMed:283398}. |
Tissue Specificity | Expressed in kidney (at protein level). {ECO:0000269|PubMed:283398}. |
Top Pathways | Valine, leucine and isoleucine degradation |
Recombinant Bovine 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial(BCKDHA) - CSB-YP002605BO CSB-EP002605BO CSB-BP002605BO CSB-MP002605BO from Cusabio
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2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial (BCKDHA), Recombinant Protein - MBS953255 from MyBioSource
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