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Protein

Pyruvate dehydrogenase protein X component, mitochondrial

Gene

Pdhx

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Required for anchoring dihydrolipoamide dehydrogenase (E3) to the dihydrolipoamide transacetylase (E2) core of the pyruvate dehydrogenase complexes of eukaryotes. This specific binding is essential for a functional PDH complex (By similarity).By similarity

GO - Molecular functioni

GO - Biological processi

Enzyme and pathway databases

ReactomeiR-MMU-204174 Regulation of pyruvate dehydrogenase (PDH) complex
R-MMU-389661 Glyoxylate metabolism and glycine degradation
R-MMU-5362517 Signaling by Retinoic Acid
R-MMU-70268 Pyruvate metabolism

Names & Taxonomyi

Protein namesi
Recommended name:
Pyruvate dehydrogenase protein X component, mitochondrial
Alternative name(s):
Dihydrolipoamide dehydrogenase-binding protein of pyruvate dehydrogenase complex
Lipoyl-containing pyruvate dehydrogenase complex component X
Gene namesi
Name:Pdhx
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi

Organism-specific databases

MGIiMGI:1351627 Pdhx

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 53MitochondrionBy similarityAdd BLAST53
ChainiPRO_000002048554 – 501Pyruvate dehydrogenase protein X component, mitochondrialAdd BLAST448

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei97N6-lipoyllysinePROSITE-ProRule annotationBy similarity1
Modified residuei194N6-acetyllysineBy similarity1
Modified residuei196PhosphoserineBy similarity1
Modified residuei394N6-succinyllysineCombined sources1

Post-translational modificationi

Delipoylated at Lys-97 by SIRT4, delipoylation decreases the PHD complex activity.By similarity

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiQ8BKZ9
MaxQBiQ8BKZ9
PaxDbiQ8BKZ9
PeptideAtlasiQ8BKZ9
PRIDEiQ8BKZ9

2D gel databases

REPRODUCTION-2DPAGEiIPI00222767

PTM databases

iPTMnetiQ8BKZ9
PhosphoSitePlusiQ8BKZ9
SwissPalmiQ8BKZ9

Expressioni

Gene expression databases

BgeeiENSMUSG00000010914
CleanExiMM_PDHX
ExpressionAtlasiQ8BKZ9 baseline and differential
GenevisibleiQ8BKZ9 MM

Interactioni

Subunit structurei

Part of the inner core of the multimeric pyruvate dehydrogenase complex that is composed of about 48 DLAT and 12 PDHX molecules. This core binds multiple copies of pyruvate dehydrogenase (subunits PDH1A and PDHB, E1), dihydrolipoamide acetyltransferase (DLAT, E2) and lipoamide dehydrogenase (DLD, E3). Interacts with SIRT4. Interacts with DLD.By similarity

Protein-protein interaction databases

BioGridi205209, 1 interactor
IntActiQ8BKZ9, 3 interactors
MINTiQ8BKZ9
STRINGi10090.ENSMUSP00000011058

Structurei

3D structure databases

ProteinModelPortaliQ8BKZ9
SMRiQ8BKZ9
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini56 – 132Lipoyl-bindingPROSITE-ProRule annotationAdd BLAST77
Domaini183 – 220Peripheral subunit-binding (PSBD)PROSITE-ProRule annotationAdd BLAST38

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Compositional biasi150 – 171Pro-richAdd BLAST22

Sequence similaritiesi

Belongs to the 2-oxoacid dehydrogenase family.Curated

Keywords - Domaini

Lipoyl, Transit peptide

Phylogenomic databases

eggNOGiKOG0557 Eukaryota
COG0508 LUCA
GeneTreeiENSGT00890000139393
HOGENOMiHOG000281566
HOVERGENiHBG005063
InParanoidiQ8BKZ9
KOiK13997
OMAiSWRLGCD
OrthoDBiEOG091G0CAV
PhylomeDBiQ8BKZ9
TreeFamiTF332256

Family and domain databases

Gene3Di3.30.559.10, 1 hit
4.10.320.10, 1 hit
InterProiView protein in InterPro
IPR003016 2-oxoA_DH_lipoyl-BS
IPR001078 2-oxoacid_DH_actylTfrase
IPR000089 Biotin_lipoyl
IPR023213 CAT-like_dom_sf
IPR036625 E3-bd_dom_sf
IPR004167 PSBD
IPR011053 Single_hybrid_motif
PfamiView protein in Pfam
PF00198 2-oxoacid_dh, 1 hit
PF00364 Biotin_lipoyl, 1 hit
PF02817 E3_binding, 1 hit
SUPFAMiSSF47005 SSF47005, 1 hit
SSF51230 SSF51230, 1 hit
PROSITEiView protein in PROSITE
PS50968 BIOTINYL_LIPOYL, 1 hit
PS00189 LIPOYL, 1 hit
PS51826 PSBD, 1 hit

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q8BKZ9-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAASWRLHCN QPLLRYLLGF SSRRSLGLAQ GAAAWPVDRG ASWRWFHSTQ
60 70 80 90 100
LLQADPIKVL MPSLSPTMEQ GNIVKWLRKE GEAVSAGDSL CEIETDKAVV
110 120 130 140 150
TLDANDDGIL AKIVVEEGAK NIQLGSLIAL MVEEGEDWKQ VEIPKDVSAP
160 170 180 190 200
PPVSKPPAPT QPSPQPQIPC PARKEHKGTA RFRLSPAARN ILEKHSLDAS
210 220 230 240 250
QGTATGPRGI FTKEDALKLV ELKQMGKITE SRPASAPPPS LSASVPPQAT
260 270 280 290 300
AGPSYPRPMT PPVSIPGQPN AAGTFTEIPA SNIRRVIAKR LTESKSTVPH
310 320 330 340 350
AYATADCDLG AVLKVRRDLV KDDIKVSVND FIIRAAAVTL KQMPGVNVTW
360 370 380 390 400
DGEGPKQLPS VDISVAVATD KGLITPIIKD AAAKGIQEIA DSVKVLSKKA
410 420 430 440 450
RDGKLMPEEY QGGSFSISNL GMFGIDEFAA VINPPQACIL AVGRFRPVLK
460 470 480 490 500
LTEDEEGNPQ LQQHQLITVT MSSDSRVVDD ELATRFLETF KANLENPMRL

G
Length:501
Mass (Da):53,999
Last modified:March 1, 2003 - v1
Checksum:i3FD1FA752EAC5092
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK047670 mRNA Translation: BAC33120.1
BC061231 mRNA Translation: AAH61231.1
CCDSiCCDS16473.1
RefSeqiNP_780303.1, NM_175094.5
UniGeneiMm.315011

Genome annotation databases

EnsembliENSMUST00000011058; ENSMUSP00000011058; ENSMUSG00000010914
GeneIDi27402
KEGGimmu:27402
UCSCiuc008lil.3 mouse

Entry informationi

Entry nameiODPX_MOUSE
AccessioniPrimary (citable) accession number: Q8BKZ9
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 4, 2005
Last sequence update: March 1, 2003
Last modified: May 23, 2018
This is version 134 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. SIMILARITY comments
    Index of protein domains and families

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