Gene Symbol | BCKDHA |
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Entrez Gene | 593 |
Alt Symbol | BCKDE1A, MSU, MSUD1, OVD1A |
Species | Human |
Gene Type | protein-coding |
Description | branched chain keto acid dehydrogenase E1, alpha polypeptide |
Other Description | 2-oxoisovalerate dehydrogenase (lipoamide)|2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial|BCKDH E1-alpha|branched-chain alpha-keto acid dehydrogenase E1 component alpha chain |
Swissprots | P12694 Q16472 B4DP47 Q16034 E7EW46 |
Accessions | AAB19267 EAW57039 EAW57040 P12694 AB209828 BAD93065 AK298188 BAG60459 AK313300 BAG36106 AL833143 CAH56215 BC007878 AAH07878 BC008933 AAH08933 BC023983 AAH23983 BE223026 BG742673 BI910860 BM702667 BQ018849 J04474 AAB59549 M22221 AAA35590 Z14093 CAA78475 NM_000709 NP_000700 NM_001164783 NP_001158255 |
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). |
Subcellular Location | Mitochondrion matrix. |
Top Pathways | Valine, leucine and isoleucine degradation |
BCKDE1A (H-5) - sc-271538 from Santa Cruz Biotechnology
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BCKDE1A (H-300) - sc-67200 from Santa Cruz Biotechnology
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BCKDE1A (G-18) - sc-51471 from Santa Cruz Biotechnology
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Anti-BCKDHA antibody - ab126173 from Abcam
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Anti-BCKDHA antibody - ab138460 from Abcam
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Anti-BCKDHA antibody - ab68094 from Abcam
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Anti-BCKDHA antibody - ab137244 from Abcam
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Anti-BCKDHA / BCKDE1A Antibody (aa71-120) - LS-C111808 from LifeSpan Bioscience
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Anti-BCKDHA / BCKDE1A Antibody (aa75-125) - LS-C290442 from LifeSpan Bioscience
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Anti-BCKDHA / BCKDE1A Antibody (aa395-445) - LS-C290444 from LifeSpan Bioscience
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Anti-BCKDHA / BCKDE1A Antibody - LS-C306521 from LifeSpan Bioscience
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Anti-BCKDHA / BCKDE1A Antibody (aa362-390) - LS-C156765 from LifeSpan Bioscience
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Anti-BCKDHA / BCKDE1A Antibody - LS-C155321 from LifeSpan Bioscience
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BCKDHA Antibody - NBP1-79616 from Novus Biologicals
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BCKDHA Antibody - NBP2-15552 from Novus Biologicals
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BCKDHA Antibody - NBP1-81022 from Novus Biologicals
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BCKDHA Antibody - H00000593-B01P from Novus Biologicals
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BCKDHA Antibody - H00000593-B01 from Novus Biologicals
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BCKDHA Antibody - H00000593-A01 from Novus Biologicals
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Anti-BCKDHA antibody produced in rabbit - HPA036640 from Sigma-Aldrich
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Anti-BCKDHA antibody produced in rabbit - SAB2702057 from Sigma-Aldrich
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Anti-BCKDHA antibody produced in mouse - SAB1405509 from Sigma-Aldrich
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Anti-BCKDHA, (N-terminal) antibody produced in rabbit - SAB2104058 from Sigma-Aldrich
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BCKDHA antibody - N-terminal region (ARP56119_P050) - ARP56119_P050 from Aviva Systems Biology
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BCKDHA-Antibody-C-term - AP6830b from Abgent, a WuXi AppTec company
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