TopFIND 4.0

Q96RR1: Twinkle protein, mitochondrial {ECO:0000305}

General Information

Protein names
- Twinkle protein, mitochondrial {ECO:0000305}
- 3.6.4.12
- Progressive external ophthalmoplegia 1 protein
- T7 gp4-like protein with intramitochondrial nucleoid localization
- T7-like mitochondrial DNA helicase
- Twinkle mtDNA helicase {ECO:0000312|HGNC:HGNC:1160}
Gene names PEO1
Organism Homo sapiens
Protease Family
Protease ID
Chromosome location
UniProt ID Q96RR1

2

N-termini

2

C-termini

0

Cleavages

0

Substrates

Sequence

        10         20         30         40         50         60 
MWVLLRSGYP LRILLPLRGE WMGRRGLPRN LAPGPPRRRY RKETLQALDM PVLPVTATEI 
        70         80         90        100        110        120 
RQYLRGHGIP FQDGHSCLRA LSPFAESSQL KGQTGVTTSF SLFIDKTTGH FLCMTSLAEG 
       130        140        150        160        170        180 
SWEDFQASVE GRGDGAREGF LLSKAPEFED SEEVRRIWNR AIPLWELPDQ EEVQLADTMF 
       190        200        210        220        230        240 
GLTKVTDDTL KRFSVRYLRP ARSLVFPWFS PGGSGLRGLK LLEAKCQGDG VSYEETTIPR 
       250        260        270        280        290        300 
PSAYHNLFGL PLISRRDAEV VLTSRELDSL ALNQSTGLPT LTLPRGTTCL PPALLPYLEQ 
       310        320        330        340        350        360 
FRRIVFWLGD DLRSWEAAKL FARKLNPKRC FLVRPGDQQP RPLEALNGGF NLSRILRTAL 
       370        380        390        400        410        420 
PAWHKSIVSF RQLREEVLGE LSNVEQAAGL RWSRFPDLNR ILKGHRKGEL TVFTGPTGSG 
       430        440        450        460        470        480 
KTTFISEYAL DLCSQGVNTL WGSFEISNVR LARVMLTQFA EGRLEDQLDK YDHWADRFED 
       490        500        510        520        530        540 
LPLYFMTFHG QQSIRTVIDT MQHAVYVYDI CHVIIDNLQF MMGHEQLSTD RIAAQDYIIG 
       550        560        570        580        590        600 
VFRKFATDNN CHVTLVIHPR KEDDDKELQT ASIFGSAKAS QEADNVLILQ DRKLVTGPGK 
       610        620        630        640        650        660 
RYLQVSKNRF DGDVGVFPLE FNKNSLTFSI PPKNKARLKK IKDDTGPVAK KPSSGKKGAT 
       670        680    
TQNSEICSGQ APTPDQPDTS KRSK

Isoforms

- Isoform 2 of Twinkle protein, mitochondrial - Isoform 3 of Twinkle protein, mitochondrial

Sequence View

        10         20         30         40         50         60 
MWVLLRSGYP LRILLPLRGE WMGRRGLPRN LAPGPPRRRY RKETLQALDM PVLPVTATEI 
        70         80         90        100        110        120 
RQYLRGHGIP FQDGHSCLRA LSPFAESSQL KGQTGVTTSF SLFIDKTTGH FLCMTSLAEG 
       130        140        150        160        170        180 
SWEDFQASVE GRGDGAREGF LLSKAPEFED SEEVRRIWNR AIPLWELPDQ EEVQLADTMF 
       190        200        210        220        230        240 
GLTKVTDDTL KRFSVRYLRP ARSLVFPWFS PGGSGLRGLK LLEAKCQGDG VSYEETTIPR 
       250        260        270        280        290        300 
PSAYHNLFGL PLISRRDAEV VLTSRELDSL ALNQSTGLPT LTLPRGTTCL PPALLPYLEQ 
       310        320        330        340        350        360 
FRRIVFWLGD DLRSWEAAKL FARKLNPKRC FLVRPGDQQP RPLEALNGGF NLSRILRTAL 
       370        380        390        400        410        420 
PAWHKSIVSF RQLREEVLGE LSNVEQAAGL RWSRFPDLNR ILKGHRKGEL TVFTGPTGSG 
       430        440        450        460        470        480 
KTTFISEYAL DLCSQGVNTL WGSFEISNVR LARVMLTQFA EGRLEDQLDK YDHWADRFED 
       490        500        510        520        530        540 
LPLYFMTFHG QQSIRTVIDT MQHAVYVYDI CHVIIDNLQF MMGHEQLSTD RIAAQDYIIG 
       550        560        570        580        590        600 
VFRKFATDNN CHVTLVIHPR KEDDDKELQT ASIFGSAKAS QEADNVLILQ DRKLVTGPGK 
       610        620        630        640        650        660 
RYLQVSKNRF DGDVGVFPLE FNKNSLTFSI PPKNKARLKK IKDDTGPVAK KPSSGKKGAT 
       670        680    
TQNSEICSGQ APTPDQPDTS KRSK         10         20         30         40         50         60 
MWVLLRSGYP LRILLPLRGE WMGRRGLPRN LAPGPPRRRY RKETLQALDM PVLPVTATEI 
        70         80         90        100        110        120 
RQYLRGHGIP FQDGHSCLRA LSPFAESSQL KGQTGVTTSF SLFIDKTTGH FLCMTSLAEG 
       130        140        150        160        170        180 
SWEDFQASVE GRGDGAREGF LLSKAPEFED SEEVRRIWNR AIPLWELPDQ EEVQLADTMF 
       190        200        210        220        230        240 
GLTKVTDDTL KRFSVRYLRP ARSLVFPWFS PGGSGLRGLK LLEAKCQGDG VSYEETTIPR 
       250        260        270        280        290        300 
PSAYHNLFGL PLISRRDAEV VLTSRELDSL ALNQSTGLPT LTLPRGTTCL PPALLPYLEQ 
       310        320        330        340        350        360 
FRRIVFWLGD DLRSWEAAKL FARKLNPKRC FLVRPGDQQP RPLEALNGGF NLSRILRTAL 
       370        380        390        400        410        420 
PAWHKSIVSF RQLREEVLGE LSNVEQAAGL RWSRFPDLNR ILKGHRKGEL TVFTGPTGSG 
       430        440        450        460        470        480 
KTTFISEYAL DLCSQGVNTL WGSFEISNVR LARVMLTQFA EGRLEDQLDK YDHWADRFED 
       490        500        510        520        530        540 
LPLYFMTFHG QQSIRTVIDT MQHAVYVYDI CHVIIDNLQF MMGHEQLSTD RIAAQDYIIG 
       550        560        570        580        590        600 
VFRKFATDNN CHVTLVIHPR KEDDDKELQT ASIFGSAKAS QEADNVLILQ DRKLVTGPGK 
       610        620        630        640        650        660 
RYLQVSKNRF DGDVGVFPLE FNKNSLTFSI PPKNKARLKK IKDDTGPVAK KPSSGKKGAT 
       670        680    
TQNSEICSGQ APTPDQPDTS KRSK



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Protein Neighborhood

Domains & Features

2 N-termini - 2 C-termini - 0 Cleavages - 0 Substrates

N-termini

C-termini

Cleavages

    Protease Position Sequence Evidence type Method Source (database) Source (Lab) Evidence name Publications (PMIDs)

Substrates

    Substrate Position Sequence Evidence type Method Source (database) Source (Lab) Evidence name Publications (PMIDs)