| Gene Symbol |
BCKDHB
|
| Entrez Gene |
594
|
| Alt Symbol |
E1B, dJ279A18.1
|
| Species |
Human
|
| Gene Type |
protein-coding
|
| Description |
branched chain keto acid dehydrogenase E1, beta polypeptide
|
| Other Description |
2-oxoisovalerate dehydrogenase beta subunit|2-oxoisovalerate dehydrogenase subunit beta, mitochondrial|BCKDE1B|BCKDH E1-beta|E1b-beta subunit of the branched-chain complex|branched chain alpha-ketoacid dehydrogenase E1-beta subunit|branched-chain alpha-keto acid dehydrogenase E1 component beta chain
|
| Swissprots |
P21953 Q5T2J3 Q9BQL0
|
| Accessions |
AAB09779 BAA14389 EAW48696 EAW48697 EAW48698 P21953 AK289977 BAF82666 AK304353 BAG65195 AK310838 AK316545 BAH14916 BC034481 AAH34481 BC040139 AAH40139 BM553932 BT020063 AAV38866 DB548202 DC372349 M55575 AAA51812 U50708 AAB16763 U51016 AAB09780 X52446 CAA36685 XM_005248756 XP_005248813 XM_006715542 XP_006715605 XM_011536023 XP_011534325 XM_011536024 XP_011534326 XM_011536025 XP_011534327 XM_011536026 XP_011534328 XM_011536027 XP_011534329 NM_000056 NP_000047 NM_183050 NP_898871
|
| 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
|