| Show obsolete/secondary identifiers |
| Mutation Data |
| LS-SNP |  | SNP Information available through LS-SNP |
| Template Structure |
|---|
| PDB | 2bff | reactivity modulation of human branched-chain alpha-ketoacid dehydrogenase by an internal molecular switch |
| DBALI | 2bffA | |
| CATH | 3.40.50.970 | Domain: 2bffA00 Rossmann fold (100%) |
| JenaImageLibrary | 2bff | |
| Target Sequence |
|---|
| UniProtKB | Q16472 | RecName: Full=2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial;EC=1.2.4.4;AltName: Full=Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain;Short=BCKDE1A;Short=BCKDH E1-al |
| InterPro | Q16472 | |
| PFAM | Q16472 | |
| Prodom | Q16472 | |
| UniProtKB | A0A024R0K3 | SubName: Full=Branched chain keto acid dehydrogenase E1, alpha polypeptide, isoform CRA_a {ECO:0000313|EMBL:EAW57039.1}; |
| InterPro | A0A024R0K3 | |
| PFAM | A0A024R0K3 | |
| Prodom | A0A024R0K3 | |
| UniProtKB | P12694 | RecName: Full=2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial;EC=1.2.4.4;AltName: Full=Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain;Short=BCKDE1A;Short=BCKDH E1-al |
| InterPro | P12694 | |
| PFAM | P12694 | |
| Prodom | P12694 | |
| UniProtKB | Q16034 | RecName: Full=2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial;EC=1.2.4.4;AltName: Full=Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain;Short=BCKDE1A;Short=BCKDH E1-al |
| InterPro | Q16034 | |
| PFAM | Q16034 | |
| Prodom | Q16034 | |
| UniProtKB | Q16034 | RecName: Full=2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial;EC=1.2.4.4;AltName: Full=Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain;Short=BCKDE1A;Short=BCKDH E1-al |
| InterPro | Q16034 | |
| PFAM | Q16034 | |
| Prodom | Q16034 | |
| UniProtKB | P12694.2 | ODBA_HUMAN RecName: Full=2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; AltName: Full=Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; Short=BCKDE1A; Short=BCKDH E1 |
| InterPro | P12694.2 | |
| PFAM | P12694.2 | |
| Prodom | P12694.2 | |
| UniProtKB | P12694 | RecName: Full=2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial;EC=1.2.4.4;AltName: Full=Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain;Short=BCKDE1A;Short=BCKDH E1-al |
| InterPro | P12694 | |
| PFAM | P12694 | |
| Prodom | P12694 | |
| UniProtKB | Q16472 | RecName: Full=2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial;EC=1.2.4.4;AltName: Full=Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain;Short=BCKDE1A;Short=BCKDH E1-al |
| InterPro | Q16472 | |
| PFAM | Q16472 | |
| Prodom | Q16472 | |
| UniProtKB | E7EW46 | RecName: Full=2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial;EC=1.2.4.4;AltName: Full=Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain;Short=BCKDE1A;Short=BCKDH E1-al |
| InterPro | E7EW46 | |
| PFAM | E7EW46 | |
| Prodom | E7EW46 | |
| UniProtKB | B4DP47 | RecName: Full=2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial;EC=1.2.4.4;AltName: Full=Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain;Short=BCKDE1A;Short=BCKDH E1-al |
| InterPro | B4DP47 | |
| PFAM | B4DP47 | |
| Prodom | B4DP47 | |
| RefSeq | NP_000700.1 | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 1 precursor [Homo sapiens] |
| GenPept | 11386135 | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial isoform 1 precursor [Homo sapiens] |
| GenPept | 568536344 | Sequence 28480 from patent US 8586006 |
| GenPept | 549828340 | Sequence 71 from patent US 8541550 |
| GenPept | 548403 | ODBA_HUMAN RecName: Full=2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial; AltName: Full=Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain; Short=BCKDE1A; Short=BCKDH E1 |
| GenPept | 18645155 | Branched chain keto acid dehydrogenase E1, alpha polypeptide |
| GenPept | 14786823 | branched chain keto acid dehydrogenase E1, alpha polypeptide (maple syrup urine disease) [Homo sapiens] |
| GenPept | 14286270 | Branched chain keto acid dehydrogenase E1, alpha polypeptide |
| GenPept | 29391 | branched chain decarboxylase alpha subunit |
| GenPept | 14043871 | Branched chain keto acid dehydrogenase E1, alpha polypeptide |
| GenPept | 353306106 | Sequence 71 from patent US 8039587 |
| GenPept | 119577444 | branched chain keto acid dehydrogenase E1, alpha polypeptide, isoform CRA_a |
| GenPept | 119577443 | branched chain keto acid dehydrogenase E1, alpha polypeptide, isoform CRA_a |
| GenPept | 358782499 | Sequence 71 from patent US 8062891 |
| GenPept | 516171720 | Sequence 71 from patent US 8470972 |
| GenPept | 534490756 | Sequence 71 from patent US 8507277 |
| GenPept | 2144336 | DEHUXA 3-methyl-2-oxobutanoate dehydrogenase (lipoamide) (EC 1.2.4.4) alpha chain precursor - human |
| GenBank | EAW57040.1 | branched chain keto acid dehydrogenase E1, alpha polypeptide, isoform CRA_a [Homo sapiens] |
| GenBank | EAW57039.1 | branched chain keto acid dehydrogenase E1, alpha polypeptide, isoform CRA_a [Homo sapiens] |
| GenBank | AAH23983.1 | Branched chain keto acid dehydrogenase E1, alpha polypeptide [Homo sapiens] |
| GenBank | AAH08933.1 | Branched chain keto acid dehydrogenase E1, alpha polypeptide [Homo sapiens] |
| GenBank | AAH07878.1 | Branched chain keto acid dehydrogenase E1, alpha polypeptide [Homo sapiens] |
| ENSEMBL | ENSP00000269980 | 2-oxoisovalerate dehydrogenase alpha subunit, mitochondrial precursor (EC 1.2.4.4) (Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain) (BCKDH E1-alpha) (BCKDE1A). [Homo sapiens] |
| EMBL | CAA78475.1 | |
| ENSEMBL | ENSP00000269980 | |
| ENSEMBL | ENSP00000269980 | TDI sequence |
| ENSEMBL | GENSCAN00000025530 | |
| ENSEMBL | ENSP00000269980 | |
| UCSCGenomeBrowser | P12694 | |
| |
