| Gene Symbol |
DBT
|
| Entrez Gene |
1629
|
| Alt Symbol |
BCATE2, BCKAD-E2, BCKADE2, BCOADC-E2, E2, E2B
|
| Species |
Human
|
| Gene Type |
protein-coding
|
| Description |
dihydrolipoamide branched chain transacylase E2
|
| Other Description |
52 kDa mitochondrial autoantigen of primary biliary cirrhosis|BCKAD E2 subunit|E2 component of branched chain alpha-keto acid dehydrogenase complex|branched chain 2-oxo-acid dehydrogenase complex component E2|branched chain acyltransferase, E2 component|branched-chain alpha-keto acid dehydrogenase complex component E2|dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex|dihydrolipoyl transacylase|dihydrolipoyllysine-residue (2-methylpropanoyl)transferase|lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial|lipoamide acyltransferase component of mitochondrial branched-chain alpha-keto acid dehydrogenase complex|mitochondrial branched chain alpha-keto acid dehydrogenase transacylase subunit (E2b)
|
| Swissprots |
B2R811 P11182 Q5VVL8
|
| Accessions |
CAA48225 EAW72963 P11182 AK024946 AK303941 BAG64869 AK313191 BAG36008 BC016675 AAH16675 BC030002 BT007372 AAP36036 DA274670 J03208 AAA35589 M19301 AAA59200 M27093 AAA64512 X57677 X66785 CAA47285 XM_005270545 XP_005270602 XM_005270546 XP_005270603 XR_946560 NM_001918 NP_001909
|
| 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). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component.
|
| Subcellular Location |
Mitochondrion matrix.
|
| Top Pathways |
Valine, leucine and isoleucine degradation
|