Overview
| Uniprot ID | P50136 |
|---|
| Protein Name | 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial |
|---|
| Gene Name | Bckdha |
|---|
| Organism | Mus musculus |
|---|
Kla Sites from experimental identification
| Position |
Flanking peptide |
| 386 |
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
MSAAKIWRPS
20
RGLRQAALLL
30
LGRSGVRGLA
40
RSHPSRQQQQ
50
QFPSLDDKPQ
60
FPGASAEFVD
70
KLEFIQPNVI
80
SGIPIYRVMD
90
RQGQIINPSE
100
DPHLPQEEVL
110
KFYRSMTLLN
120
TMDRILYESQ
130
REGRISFYMT
140
NYGEEGTHVG
150
SAAALERTDL
160
VFGQYREAGV
170
LMYRDYPLEL
180
FMSQCYGNVN
190
DPGKGRQMPV
200
HYGCKERHFV
210
TISSPLATQI
220
PQAVGAAYAA
230
KRANANRIVI
240
CYFGEGAASE
250
GDAHAGFNFA
260
ATLECPIIFF
270
CRNNGYAIST
280
PTSEQYRGDG
290
IAARGPGYGI
300
KSIRVDGNDV
310
FAVYNATKEA
320
RRRAVAENQP
330
FLIEAMTYRI
340
GHHSTSDDSS
350
AYRSVDEVNY
360
WDKQDHPISR
370
LRQYLLNQGW
380
WDEEQEKAWR
390
KQSRKKVMEA
400
FEQAERKLKP
410
NPSLLFSDVY
420
QEMPAQLRRQ
430
QESLARHLQT
440
YGEHYPLDHF
EK
Gene Ontology
| Classification |
GO ID |
Description |
| Cellular Component |
GO:0160157 |
branched-chain alpha-ketoacid dehydrogenase complex |
| Cellular Component |
GO:0005759 |
mitochondrial matrix |
| Cellular Component |
GO:0005739 |
mitochondrion |
| 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 |
Reference
[1] Zhuo W, Zhang M, Tan J, Gao Y, Wang Y et al.. Lysine lactylation analysis of proteins in the heart of the Kawasaki disease mouse model.. Front Cell Dev Biol 13:1550220. 2025. PMID: 40114965.