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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)LengthDescriptionGraphical viewFeature identifierActions
Nucleotide bindingi416 – 4238ATPPROSITE-ProRule annotation

GO - Molecular functioni

  1. 5'-3' DNA helicase activity Source: UniProtKB
  2. ATP binding Source: UniProtKB-KW
  3. DNA helicase activity Source: MGI
  4. protease binding Source: MGI
  5. single-stranded DNA binding Source: UniProtKB

GO - Biological processi

  1. DNA unwinding involved in DNA replication Source: MGI
  2. mitochondrial DNA replication Source: UniProtKB
  3. protein hexamerization Source: MGI
  4. protein homooligomerization Source: MGI
  5. transcription from mitochondrial promoter Source: MGI
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
ProteomesiUP000000589 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

  1. mitochondrial nucleoid Source: UniProtKB
  2. mitochondrion Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion, Mitochondrion nucleoid

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3131MitochondrionBy similarityAdd
BLAST
Chaini32 – 685654Twinkle protein, mitochondrialPRO_0000042641Add
BLAST

Proteomic databases

MaxQBiQ8CIW5.
PaxDbiQ8CIW5.
PRIDEiQ8CIW5.

PTM databases

PhosphoSiteiQ8CIW5.

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

BgeeiQ8CIW5.
CleanExiMM_PEO1.
ExpressionAtlasiQ8CIW5. baseline and differential.
GenevestigatoriQ8CIW5.

Interactioni

Subunit structurei

Forms multimers in vitro, including hexamers.By similarity

Structurei

3D structure databases

ProteinModelPortaliQ8CIW5.
SMRiQ8CIW5. Positions 260-626.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini385 – 636252SF4 helicasePROSITE-ProRule annotationAdd
BLAST

Coiled coil

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Coiled coili454 – 48229Sequence AnalysisAdd
BLAST

Sequence similaritiesi

Contains 1 SF4 helicase domain.PROSITE-ProRule annotation

Keywords - Domaini

Coiled coil, Transit peptide

Phylogenomic databases

eggNOGiNOG29349.
GeneTreeiENSGT00390000004495.
HOGENOMiHOG000273872.
HOVERGENiHBG059782.
InParanoidiQ8CIW5.
KOiK17680.
OMAiETTIPRP.
OrthoDBiEOG75MVVT.
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)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei1 – 398398Missing in isoform 2. 1 PublicationVSP_015962Add
BLAST
Alternative sequencei399 – 41517LNRLL…LTVFT → MCCQTLLLTQSFVRPPI in isoform 2. 1 PublicationVSP_015963Add
BLAST

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. Positions 260-626.
ModBaseiSearch...
MobiDBiSearch...

PTM databases

PhosphoSiteiQ8CIW5.

Proteomic databases

MaxQBiQ8CIW5.
PaxDbiQ8CIW5.
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

eggNOGiNOG29349.
GeneTreeiENSGT00390000004495.
HOGENOMiHOG000273872.
HOVERGENiHBG059782.
InParanoidiQ8CIW5.
KOiK17680.
OMAiETTIPRP.
OrthoDBiEOG75MVVT.
PhylomeDBiQ8CIW5.
TreeFamiTF105994.

Miscellaneous databases

NextBioi378032.
PROiQ8CIW5.
SOURCEiSearch...

Gene expression databases

BgeeiQ8CIW5.
CleanExiMM_PEO1.
ExpressionAtlasiQ8CIW5. baseline and differential.
GenevestigatoriQ8CIW5.

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...

Publicationsi

« Hide 'large scale' publications
  1. "Cloning of full length Mus musculus Twinkle cDNA."
    Spelbrink J.N.
    Submitted (SEP-2001) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
    Tissue: Fibroblast.
  2. "The transcriptional landscape of the mammalian genome."
    Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J.
    , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y.
    Science 309:1559-1563(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
    Strain: BALB/c.
  3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 2).
    Strain: FVB/N.
    Tissue: Liver.
  4. Cited for: TISSUE SPECIFICITY, COREGULATION WITH MRPL43, FUNCTION.

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: February 4, 2015
This is version 96 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

External Data

Dasty 3

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 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.