| Gene Symbol |
BCKDHA
|
| 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
|