| Gene Symbol |
BCKDHA
|
| Entrez Gene |
102134149
|
| Alt Symbol |
BCKDE1A
|
| Species |
Monkey
|
| Gene Type |
protein-coding
|
| Description |
branched chain keto acid dehydrogenase E1, alpha polypeptide
|
| Other Description |
2-oxoisovalerate dehydrogenase alpha subunit|2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial|BCKDH E1-alpha|Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain
|
| Swissprots |
Q8HXY4
|
| Accessions |
Q8HXY4 AB083305 BAC20584 NM_001284089 NP_001271018 XM_005589354 XP_005589411
|
| 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 {ECO:0000250}.
|
| Top Pathways |
Valine, leucine and isoleucine degradation
|