Overview
| Uniprot ID | F7EV94 |
|---|
| Protein Name | 2-oxoisovalerate dehydrogenase subunit alpha |
|---|
| Gene Name | Bckdha |
|---|
| Organism | Rattus norvegicus |
|---|
Kla Sites from experimental identification
| Position |
Flanking peptide |
| 413 |
WRKQSRKKVMEAFEQ |
Function
Together with BCKDHB forms the heterotetrameric E1 subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex. The BCKD complex catalyzes the multi-step oxidative decarboxylation of alpha-ketoacids derived from the branched-chain amino-acids valine, leucine and isoleucine producing CO2 and acyl-CoA which is subsequently utilized to produce energy. The E1 subunit catalyzes the first step with the decarboxylation of the alpha-ketoacid forming an enzyme-product intermediate. A reductive acylation mediated by the lipoylamide cofactor of E2 extracts the acyl group from the E1 active site for the next step of the reaction
Protein Sequence
10
MGQTHVYLLR
20
KEEWLLAKMA
30
VAMSAAKIWR
40
PSRGLRQAAL
50
LLLGRPGARG
60
LARFHPSRQQ
70
QQQFPSLDDK
80
PQFPGASAEF
90
VDKLEFIQPN
100
VISGIPIYRV
110
MDRQGQIINP
120
SEDPHLPQEE
130
VLKLYRSMTL
140
LNTMDRILYE
150
SQRQGRISFY
160
MTNYGEEGTH
170
VGSAAALERT
180
DLVFGQYREA
190
GVLMYRDYPL
200
ELFMAQCYGN
210
VSDPGKGRQM
220
PVHYGCKERH
230
FVTISSPLAT
240
QIPQAVGAAY
250
AAKRANANQI
260
VICYFGEGAA
270
SEGDAHAGFN
280
FAATLECPII
290
FFCRNNGYAI
300
STPTSEQYRG
310
DGIAARGPGY
320
GIMSIRVDGN
330
DVFAVYNATK
340
EARRRAVAEN
350
QPFLIETCLL
360
PHLHSLPCAT
370
SLLDDSSAYR
380
SVDEVNYWDK
390
QDHPISRLRQ
400
YLLNQGWWDE
410
EQEKAWRKQS
420
RKKVMEAFEQ
430
AERKLKPNPS
440
LLFSDVYQEM
450
PAQLRRQQES
460
LARHLQTYGE
HYPLDHFDK
Gene Ontology
| Classification |
GO ID |
Description |
| Cellular Component |
GO:0005759 |
mitochondrial matrix |
| Molecular Function |
GO:0003863 |
branched-chain 2-oxo acid dehydrogenase activity |
| Molecular Function |
GO:0046872 |
metal ion binding |
Reference
[1] Chen Y, Sun W, Sun Z, Zhao H, Wu T et al.. Effect of electroacupuncture on hippocampal protein lactylation in a rat model of vascular dementia.. Front Neurol 16:1629474. 2025. PMID: 40963935.