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Q8CIW5

- PEO1_MOUSE

UniProt

Q8CIW5 - PEO1_MOUSE

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Protein

Twinkle protein, mitochondrial

Gene

Peo1

Organism
Mus musculus (Mouse)
Status
Reviewed - 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. 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: Ensembl
  4. protein homooligomerization Source: Ensembl
  5. transcription from mitochondrial promoter Source: Ensembl
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: 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.
OMAiGSWEDFQ.
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. Align

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:
EMBL
GenBank
DDBJ
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:
EMBL
GenBank
DDBJ
Links Updated
AY059385 mRNA. Translation: AAL27647.1 .
AK146244 mRNA. Translation: BAE27008.1 .
BC034909 mRNA. Translation: AAH34909.1 .
BC071195 mRNA. Translation: AAH71195.1 .
CCDSi CCDS29854.1. [Q8CIW5-1 ]
RefSeqi NP_722491.2. NM_153796.3. [Q8CIW5-1 ]
UniGenei Mm.105585.

3D structure databases

ProteinModelPortali Q8CIW5.
SMRi Q8CIW5. Positions 260-626.
ModBasei Search...
MobiDBi Search...

PTM databases

PhosphoSitei Q8CIW5.

Proteomic databases

MaxQBi Q8CIW5.
PaxDbi Q8CIW5.
PRIDEi Q8CIW5.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENSMUST00000026227 ; ENSMUSP00000026227 ; ENSMUSG00000025209 . [Q8CIW5-1 ]
GeneIDi 226153.
KEGGi mmu:226153.
UCSCi uc008hqh.1. mouse. [Q8CIW5-2 ]
uc008hqi.1. mouse. [Q8CIW5-1 ]

Organism-specific databases

CTDi 226153.
MGIi MGI:2137410. Peo1.

Phylogenomic databases

eggNOGi NOG29349.
GeneTreei ENSGT00390000004495.
HOGENOMi HOG000273872.
HOVERGENi HBG059782.
InParanoidi Q8CIW5.
KOi K17680.
OMAi GSWEDFQ.
OrthoDBi EOG75MVVT.
PhylomeDBi Q8CIW5.
TreeFami TF105994.

Miscellaneous databases

NextBioi 378032.
PROi Q8CIW5.
SOURCEi Search...

Gene expression databases

Bgeei Q8CIW5.
CleanExi MM_PEO1.
ExpressionAtlasi Q8CIW5. baseline and differential.
Genevestigatori Q8CIW5.

Family and domain databases

Gene3Di 3.40.50.300. 1 hit.
InterProi IPR007694. DNA_helicase_DnaB-like_C.
IPR027417. P-loop_NTPase.
IPR027032. Twinkle-like.
[Graphical view ]
PANTHERi PTHR12873. PTHR12873. 1 hit.
SUPFAMi SSF52540. SSF52540. 1 hit.
PROSITEi PS51199. SF4_HELICASE. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "Cloning of full length Mus musculus Twinkle cDNA."
    Spelbrink J.N.
    Submitted (OCT-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(2005) [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(2004) [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: October 29, 2014
This is version 94 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