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Protein

Twinkle protein, mitochondrial

Gene

Peo1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at transcript leveli

Functioni

Involved in mitochondrial DNA (mtDNA) metabolism. Could function as an adenine nucleotide-dependent DNA helicase. Function infered to be critical for lifetime maintenance of mtDNA integrity. May be a key regulator of mtDNA copy number in mammals.1 Publication

Catalytic activityi

ATP + H2O = ADP + phosphate.

Regions

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Nucleotide bindingi416 – 423ATPPROSITE-ProRule annotation8

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Helicase, Hydrolase

Keywords - Biological processi

DNA replication

Keywords - Ligandi

ATP-binding, Nucleotide-binding

Names & Taxonomyi

Protein namesi
Recommended name:
Twinkle protein, mitochondrial (EC:3.6.4.12)
Alternative name(s):
Progressive external ophthalmoplegia 1 protein homolog
T7 gp4-like protein with intramitochondrial nucleoid localization
T7-like mitochondrial DNA helicase
Gene namesi
Name:Peo1
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 19

Organism-specific databases

MGIiMGI:2137410. Peo1.

Subcellular locationi

  • Mitochondrion matrixmitochondrion nucleoid By similarity

  • Note: Colocalizes with mtDNA in mitochondrial nucleoids, a nucleoproteins complex consisting of a number of copies of proteins associated with mtDNA, probably involved in mtDNA maintenance and expression.By similarity

GO - Cellular componenti

  • mitochondrial nucleoid Source: UniProtKB
  • mitochondrion Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion, Mitochondrion nucleoid

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 31MitochondrionBy similarityAdd BLAST31
ChainiPRO_000004264132 – 685Twinkle protein, mitochondrialAdd BLAST654

Proteomic databases

EPDiQ8CIW5.
PaxDbiQ8CIW5.
PeptideAtlasiQ8CIW5.
PRIDEiQ8CIW5.

PTM databases

iPTMnetiQ8CIW5.
PhosphoSitePlusiQ8CIW5.

Expressioni

Tissue specificityi

Ubiquitous with the highest levels in the liver, heart and kidneys. The skeletal muscle, brain and testis showed lower but detectable expression. Expression is coregulated with MRPL43.1 Publication

Gene expression databases

BgeeiENSMUSG00000025209.
CleanExiMM_PEO1.
GenevisibleiQ8CIW5. MM.

Interactioni

Subunit structurei

Forms multimers in vitro, including hexamers.By similarity

GO - Molecular functioni

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000026227.

Structurei

3D structure databases

ProteinModelPortaliQ8CIW5.
SMRiQ8CIW5.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini385 – 636SF4 helicasePROSITE-ProRule annotationAdd BLAST252

Coiled coil

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Coiled coili454 – 482Sequence analysisAdd BLAST29

Sequence similaritiesi

Contains 1 SF4 helicase domain.PROSITE-ProRule annotation

Keywords - Domaini

Coiled coil, Transit peptide

Phylogenomic databases

eggNOGiKOG2373. Eukaryota.
ENOG410XRZX. LUCA.
GeneTreeiENSGT00390000004495.
HOGENOMiHOG000273872.
HOVERGENiHBG059782.
InParanoidiQ8CIW5.
KOiK17680.
OMAiLPLRGEW.
OrthoDBiEOG091G03Y8.
PhylomeDBiQ8CIW5.
TreeFamiTF105994.

Family and domain databases

Gene3Di3.40.50.300. 1 hit.
InterProiIPR007694. DNA_helicase_DnaB-like_C.
IPR027417. P-loop_NTPase.
IPR027032. Twinkle-like.
[Graphical view]
PANTHERiPTHR12873. PTHR12873. 1 hit.
SUPFAMiSSF52540. SSF52540. 1 hit.
PROSITEiPS51199. SF4_HELICASE. 1 hit.
[Graphical view]

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: Q8CIW5-1) [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
MWLLLRRAYP LRILLPLRGE WVGRRGLPRS LAPGPPRRRY RKEALPALEM
60 70 80 90 100
PVSPVTTTEI RQYLRAHGIP FQDGHSCLRA PSPFVVSSDI KNEKKDAPTS
110 120 130 140 150
FCLFIDKTTG HFLCMTSLAE GSWEDLQASV EGRGDGAKEG VLLREGPEAE
160 170 180 190 200
VREEVLRIWN RAIPLWELPD PEEAQLARVM FGLTKVTDDT LRRFSVRYLR
210 220 230 240 250
SARSLVFPWF TPGSSGLRGL KLLGAEGQEN GVQYVETTIP RPGVYHNLFG
260 270 280 290 300
LPLISRRDTE VVVTSRELDS LALSQSTGLP TLSLPRGTVC LPPALLPYLE
310 320 330 340 350
QFRRIVFWLG DDLRSWEAAK LFARKLNPKR CSLVRPGNQQ PRPLEALNQG
360 370 380 390 400
LSLPRILRTA LPAWHKSIVS FRQLREEVLG ELSNVEQAAG VRWSRFPDLN
410 420 430 440 450
RLLKGHRKGE LTVFTGPTGS GKTTFISEYA LDLCTQGVNT LWGSFEISNV
460 470 480 490 500
RLARVMLTQF AVTRLEEQLD KYEEWADRFE DLPLYFMTFH GQQSIRSVID
510 520 530 540 550
TMQHAVYVYD VCHVVIDNLQ FMMGHEQLSS DRIAAQDYIV GAFRKFATDN
560 570 580 590 600
SCHVTLVIHP RKEDDDKELQ TASIFGSAKA SQEADNVLIL QDRKLVTGPG
610 620 630 640 650
KRYLQVSKNR FDGDVGVFPL EFNKNSLTFS IPPKSKARLK KIKDDNGLVA
660 670 680
KKSSSGKKGA AHQNPEICLG QDPSPAQPDT SKSSG
Length:685
Mass (Da):76,993
Last modified:March 1, 2003 - v1
Checksum:iF9CF69BDC4DCF838
GO
Isoform 2 (identifier: Q8CIW5-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-398: Missing.
     399-415: LNRLLKGHRKGELTVFT → MCCQTLLLTQSFVRPPI

Show »
Length:287
Mass (Da):32,016
Checksum:iE636DE51D48B31D3
GO

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0159621 – 398Missing in isoform 2. 1 PublicationAdd BLAST398
Alternative sequenceiVSP_015963399 – 415LNRLL…LTVFT → MCCQTLLLTQSFVRPPI in isoform 2. 1 PublicationAdd BLAST17

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY059385 mRNA. Translation: AAL27647.1.
AK146244 mRNA. Translation: BAE27008.1.
BC034909 mRNA. Translation: AAH34909.1.
BC071195 mRNA. Translation: AAH71195.1.
CCDSiCCDS29854.1. [Q8CIW5-1]
RefSeqiNP_722491.2. NM_153796.3. [Q8CIW5-1]
UniGeneiMm.105585.

Genome annotation databases

EnsembliENSMUST00000026227; ENSMUSP00000026227; ENSMUSG00000025209. [Q8CIW5-1]
GeneIDi226153.
KEGGimmu:226153.
UCSCiuc008hqh.1. mouse. [Q8CIW5-2]
uc008hqi.1. mouse. [Q8CIW5-1]

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY059385 mRNA. Translation: AAL27647.1.
AK146244 mRNA. Translation: BAE27008.1.
BC034909 mRNA. Translation: AAH34909.1.
BC071195 mRNA. Translation: AAH71195.1.
CCDSiCCDS29854.1. [Q8CIW5-1]
RefSeqiNP_722491.2. NM_153796.3. [Q8CIW5-1]
UniGeneiMm.105585.

3D structure databases

ProteinModelPortaliQ8CIW5.
SMRiQ8CIW5.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10090.ENSMUSP00000026227.

PTM databases

iPTMnetiQ8CIW5.
PhosphoSitePlusiQ8CIW5.

Proteomic databases

EPDiQ8CIW5.
PaxDbiQ8CIW5.
PeptideAtlasiQ8CIW5.
PRIDEiQ8CIW5.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000026227; ENSMUSP00000026227; ENSMUSG00000025209. [Q8CIW5-1]
GeneIDi226153.
KEGGimmu:226153.
UCSCiuc008hqh.1. mouse. [Q8CIW5-2]
uc008hqi.1. mouse. [Q8CIW5-1]

Organism-specific databases

CTDi226153.
MGIiMGI:2137410. Peo1.

Phylogenomic databases

eggNOGiKOG2373. Eukaryota.
ENOG410XRZX. LUCA.
GeneTreeiENSGT00390000004495.
HOGENOMiHOG000273872.
HOVERGENiHBG059782.
InParanoidiQ8CIW5.
KOiK17680.
OMAiLPLRGEW.
OrthoDBiEOG091G03Y8.
PhylomeDBiQ8CIW5.
TreeFamiTF105994.

Miscellaneous databases

PROiQ8CIW5.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000025209.
CleanExiMM_PEO1.
GenevisibleiQ8CIW5. MM.

Family and domain databases

Gene3Di3.40.50.300. 1 hit.
InterProiIPR007694. DNA_helicase_DnaB-like_C.
IPR027417. P-loop_NTPase.
IPR027032. Twinkle-like.
[Graphical view]
PANTHERiPTHR12873. PTHR12873. 1 hit.
SUPFAMiSSF52540. SSF52540. 1 hit.
PROSITEiPS51199. SF4_HELICASE. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiPEO1_MOUSE
AccessioniPrimary (citable) accession number: Q8CIW5
Secondary accession number(s): Q8K1Z1
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 25, 2005
Last sequence update: March 1, 2003
Last modified: November 2, 2016
This is version 106 of the entry and version 1 of the sequence. [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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.