| Gene Symbol |
BCKDHA
|
| 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
|