Overview
| Uniprot ID | P11960 |
|---|
| Protein Name | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial |
|---|
| Gene Name | Bckdha |
|---|
| Organism | Rattus norvegicus |
|---|
Kla Sites from experimental identification
| Position |
Flanking peptide |
| 385 |
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
SAAKIWRPSR
20
GLRQAALLLL
30
GRPGARGLAR
40
FHPSRQQQQQ
50
FPSLDDKPQF
60
PGASAEFVDK
70
LEFIQPNVIS
80
GIPIYRVMDR
90
QGQIINPSED
100
PHLPQEEVLK
110
LYRSMTLLNT
120
MDRILYESQR
130
QGRISFYMTN
140
YGEEGTHVGS
150
AAALERTDLV
160
FGQYREAGVL
170
MYRDYPLELF
180
MAQCYGNVSD
190
PGKGRQMPVH
200
YGCKERHFVT
210
ISSPLATQIP
220
QAVGAAYAAK
230
RANANQIVIC
240
YFGEGAASEG
250
DAHAGFNFAA
260
TLECPIIFFC
270
RNNGYAISTP
280
TSEQYRGDGI
290
AARGPGYGIM
300
SIRVDGNDVF
310
AVYNATKEAR
320
RRAVAENQPF
330
LIEAMTYRIG
340
HHSTSDDSSA
350
YRSVDEVNYW
360
DKQDHPISRL
370
RQYLLNQGWW
380
DEEQEKAWRK
390
QSRKKVMEAF
400
EQAERKLKPN
410
PSLLFSDVYQ
420
EMPAQLRRQQ
430
ESLARHLQTY
440
GEHYPLDHFD
K
Gene Ontology
| Classification |
GO ID |
Description |
| Cellular Component |
GO:0160157 |
branched-chain alpha-ketoacid dehydrogenase complex |
| Cellular Component |
GO:0005759 |
mitochondrial matrix |
| Molecular Function |
GO:0003863 |
branched-chain 2-oxo acid dehydrogenase activity |
| Molecular Function |
GO:0046872 |
metal ion binding |
| Molecular Function |
GO:0044877 |
protein-containing complex binding |
| Biological Process |
GO:0120552 |
branched-chain alpha-keto acid decarboxylation to branched-chain acyl-CoA |
| Biological Process |
GO:0009083 |
branched-chain amino acid catabolic process |
| Biological Process |
GO:0051591 |
response to cAMP |
| Biological Process |
GO:0051384 |
response to glucocorticoid |
| Biological Process |
GO:0007584 |
response to nutrient |
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.