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Protein

Pyruvate dehydrogenase phosphatase regulatory subunit, mitochondrial

Gene

PDPR

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Decreases the sensitivity of PDP1 to magnesium ions, and this inhibition is reversed by the polyamine spermine.By similarity

Caution

It is uncertain whether Met-1 or Met-2 is the initiator.Curated

GO - Molecular functioni

GO - Biological processi

Enzyme and pathway databases

ReactomeiR-HSA-204174 Regulation of pyruvate dehydrogenase (PDH) complex

Names & Taxonomyi

Protein namesi
Recommended name:
Pyruvate dehydrogenase phosphatase regulatory subunit, mitochondrial
Short name:
PDPr
Gene namesi
Name:PDPR
Synonyms:KIAA1990
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 16

Organism-specific databases

EuPathDBiHostDB:ENSG00000090857.13
HGNCiHGNC:30264 PDPR
MIMi617835 gene
neXtProtiNX_Q8NCN5

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

Pathology & Biotechi

Organism-specific databases

OpenTargetsiENSG00000090857

Polymorphism and mutation databases

BioMutaiPDPR
DMDMi182668644

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 27MitochondrionSequence analysisAdd BLAST27
ChainiPRO_000032874028 – 879Pyruvate dehydrogenase phosphatase regulatory subunit, mitochondrialAdd BLAST852

Proteomic databases

EPDiQ8NCN5
MaxQBiQ8NCN5
PaxDbiQ8NCN5
PeptideAtlasiQ8NCN5
PRIDEiQ8NCN5

PTM databases

iPTMnetiQ8NCN5
PhosphoSitePlusiQ8NCN5

Expressioni

Gene expression databases

BgeeiENSG00000090857
ExpressionAtlasiQ8NCN5 baseline and differential
GenevisibleiQ8NCN5 HS

Organism-specific databases

HPAiHPA048092
HPA070386

Interactioni

Subunit structurei

Heterodimer of a catalytic (PDP1) and a regulatory (PDPR) subunit.By similarity

Protein-protein interaction databases

BioGridi120384, 30 interactors
IntActiQ8NCN5, 3 interactors
STRINGi9606.ENSP00000288050

Structurei

3D structure databases

ProteinModelPortaliQ8NCN5
SMRiQ8NCN5
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the GcvT family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2844 Eukaryota
COG0404 LUCA
GeneTreeiENSGT00530000063120
HOGENOMiHOG000251716
HOVERGENiHBG103854
InParanoidiQ8NCN5
KOiK17509
OMAiSMYTAIC
OrthoDBiEOG091G01FW
PhylomeDBiQ8NCN5
TreeFamiTF314735

Family and domain databases

Gene3Di3.30.1360.120, 2 hits
3.50.50.60, 3 hits
InterProiView protein in InterPro
IPR006076 FAD-dep_OxRdtase
IPR036188 FAD/NAD-bd_sf
IPR032503 FAO_M
IPR013977 GCV_T_C
IPR006222 GCV_T_N
IPR029043 GcvT/YgfZ_C
IPR027266 TrmE/GcvT_dom1
PfamiView protein in Pfam
PF01266 DAO, 1 hit
PF16350 FAO_M, 1 hit
PF01571 GCV_T, 1 hit
PF08669 GCV_T_C, 1 hit
SUPFAMiSSF101790 SSF101790, 1 hit
SSF51905 SSF51905, 2 hits

Sequences (2)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q8NCN5-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MMFYRLLSIV GRQRASPGWQ NWSSARNSTS AAEARSMALP TQAQVVICGG
60 70 80 90 100
GITGTSVAYH LSKMGWKDIV LLEQGRLAAG STRFCAGILS TARHLTIEQK
110 120 130 140 150
MADYSNKLYY QLEQETGIQT GYTRTGSIFL AQTQDRLISL KRINAGLNVI
160 170 180 190 200
GIPSEIISPK KVAELHHLLN VHDLVGAMHV PEDAVVSSAD VALALASAAS
210 220 230 240 250
QNGVQIYDRT SVLHVMVKKG QVTGVETDKG QIECQYFVNC AGQWAYELGL
260 270 280 290 300
SNEEPVSIPL HACEHFYLLT RPLETPLQSS TPTIVDADGR IYIRNWQGGI
310 320 330 340 350
LSGGFEKNPK PIFTEGKNQL EIQNLQEDWD HFEPLLSSLL RRMPELETLE
360 370 380 390 400
IMKLVNCPET FTPDMRCIMG ESPAVQGYFV LAGMNSAGLS FGGGAGKYLA
410 420 430 440 450
EWMVHGYPSE NVWELDLKRF GALQSSRTFL RHRVMEVMPL MYDLKVPRWD
460 470 480 490 500
FQTGRQLRTS PLYDRLDAQG ARWMEKHGFE RPKYFVPPDK DLLALEQSKT
510 520 530 540 550
FYKPDWFDIV ESEVKCCKEA VCVIDMSSFT KFEITSTGDQ ALEVLQYLFS
560 570 580 590 600
NDLDVPVGHI VHTGMLNEGG GYENDCSIAR LNKRSFFMIS PTDQQVHCWA
610 620 630 640 650
WLKKHMPKDS NLLLEDVTWK YTALNLIGPR AVDVLSELSY APMTPDHFPS
660 670 680 690 700
LFCKEMSVGY ANGIRVMSMT HTGEPGFMLY IPIEYALHVY NEVMSVGQKY
710 720 730 740 750
GIRNAGYYAL RSLRIEKFFA FWGQDINNLT TPLECGRESR VKLEKGMDFI
760 770 780 790 800
GRDALLQQKQ NGVYKRLTMF ILDDHDSDLD LWPWWGEPIY RNGQYVGKTT
810 820 830 840 850
SSAYSYSLER HVCLGFVHNF SEDTGEEQVV TADFINRGEY EIDIAGYRFQ
860 870
AKAKLYPVAS LFTQKRRKDD MELSDLHGK
Length:879
Mass (Da):99,364
Last modified:April 8, 2008 - v2
Checksum:iD555CDE2A289753F
GO
Isoform 2 (identifier: Q8NCN5-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-655: Missing.

Note: No experimental confirmation available.
Show »
Length:224
Mass (Da):25,877
Checksum:iBDC4BBA660C3D86A
GO

Sequence cautioni

The sequence BAC02699 differs from that shown. Reason: Erroneous initiation.Curated
The sequence BAF85191 differs from that shown. Reason: Erroneous initiation.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti29T → A in CAH10555 (PubMed:17974005).Curated1
Sequence conflicti47I → V in CAH10555 (PubMed:17974005).Curated1
Sequence conflicti59 – 61YHL → HHQ in CAH10555 (PubMed:17974005).Curated3
Sequence conflicti110Y → H in BAF85191 (PubMed:14702039).Curated1
Sequence conflicti110Y → H in CAH10555 (PubMed:17974005).Curated1
Sequence conflicti171V → A in CAH10555 (PubMed:17974005).Curated1
Sequence conflicti206I → V in CAH10555 (PubMed:17974005).Curated1
Sequence conflicti210T → A in BAF85191 (PubMed:14702039).Curated1
Sequence conflicti284I → T in CAH10494 (PubMed:17974005).Curated1
Sequence conflicti355V → A in CAH10494 (PubMed:17974005).Curated1
Sequence conflicti363P → S in CAH10555 (PubMed:17974005).Curated1
Sequence conflicti497Q → R in CAH10494 (PubMed:17974005).Curated1
Sequence conflicti597H → D in BAF85191 (PubMed:14702039).Curated1
Sequence conflicti662N → K in CAH10555 (PubMed:17974005).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_042504109Y → H. Corresponds to variant dbSNP:rs2549532Ensembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0560771 – 655Missing in isoform 2. 1 PublicationAdd BLAST655

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB082521 mRNA Translation: BAC02699.1 Different initiation.
AK093391 mRNA Translation: BAG52703.1
AK292502 mRNA Translation: BAF85191.1 Different initiation.
BX538153 mRNA Translation: CAH10555.1
CR627404 mRNA Translation: CAH10494.1
AC009022 Genomic DNA No translation available.
AC009060 Genomic DNA No translation available.
BC150251 mRNA Translation: AAI50252.1
CCDSiCCDS45520.1 [Q8NCN5-1]
RefSeqiNP_001309046.1, NM_001322117.1 [Q8NCN5-1]
NP_060460.4, NM_017990.4 [Q8NCN5-1]
XP_005256072.1, XM_005256015.2 [Q8NCN5-1]
XP_005256073.1, XM_005256016.3 [Q8NCN5-1]
XP_016878876.1, XM_017023387.1 [Q8NCN5-1]
UniGeneiHs.744043

Genome annotation databases

EnsembliENST00000288050; ENSP00000288050; ENSG00000090857 [Q8NCN5-1]
ENST00000568530; ENSP00000457916; ENSG00000090857 [Q8NCN5-1]
GeneIDi55066
KEGGihsa:55066
UCSCiuc002eyf.2 human [Q8NCN5-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar proteinsi

Entry informationi

Entry nameiPDPR_HUMAN
AccessioniPrimary (citable) accession number: Q8NCN5
Secondary accession number(s): A7E298
, A8K8Y7, B3KSE1, Q6AI20, Q6AWC9
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 8, 2008
Last sequence update: April 8, 2008
Last modified: May 23, 2018
This is version 132 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

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