Twinkle mtDNA helicase (EC 3.6.4.12) (Progressive external ophthalmoplegia 1 protein) (T7 gp4-like protein with intramitochondrial nucleoid localization) (T7-like mitochondrial DNA helicase) (Twinkle protein, mitochondrial)
1_MWVLL 6_ RSGYP 11_ LRILL 16_ PLRGE 21_ WMGRR 26_ GLPRN 31_ LAPGP 36_ PRRRY 41_ RKETL 46_ QALDM 51_ PVLPV 56_ TATEI 61_ RQYLR 66_ GHGIP 71_ FQDGH 76_ SCLRA 81_ LSPFA 86_ ESSQL 91_ KGQTG 96_ VTTSF 101_ SLFID 106_ KTTGH 111_ FLCMT 116_ SLAEG 121_ SWEDF 126_ QASVE 131_ GRGDG 136_ AREGF 141_ LLSKA 146_ PEFED 151_ SEEVR 156_ RIWNR 161_ AIPLW 166_ ELPDQ 171_ EEVQL 176_ ADTMF 181_ GLTKV 186_ TDDTL 191_ KRFSV 196_ RYLRP 201_ ARSLV 206_ FPWFS 211_ PGGSG 216_ LRGLK 221_ LLEAK 226_ CQGDG 231_ VSYEE 236_ TTIPR 241_ PSAYH 246_ NLFGL 251_ PLISR 256_ RDAEV 261_ VLTSR 266_ ELDSL 271_ ALNQS 276_ TGLPT 281_ LTLPR 286_ GTTCL 291_ PPALL 296_ PYLEQ 301_ FRRIV 306_ FWLGD 311_ DLRSW 316_ EAAKL 321_ FARKL 326_ NPKRC 331_ FLVRP 336_ GDQQP 341_ RPLEA 346_ LNGGF 351_ NLSRI 356_ LRTAL 361_ PAWHK 366_ SIVSF 371_ RQLRE 376_ EVLGE 381_ LSNVE 386_ QAAGL 391_ RWSRF 396_ PDLNR 401_ ILKGH 406_ RKGEL 411_ TVFTG 416_ PTGSG 421_ KTTFI 426_ SEYAL 431_ DLCSQ 436_ GVNTL 441_ WGSFE 446_ ISNVR 451_ LARVM 456_ LTQFA 461_ EGRLE 466_ DQLDK 471_ YDHWA 476_ DRFED 481_ LPLYF 486_ MTFHG 491_ QQSIR 496_ TVIDT 501_ MQHAV 506_ YVYDI 511_ CHVII 516_ DNLQF 521_ MMGHE 526_ QLSTD 531_ RIAAQ 536_ DYIIG 541_ VFRKF 546_ ATDNN 551_ CHVTL 556_ VIHPR 561_ KEDDD 566_ KELQT 571_ ASIFG 576_ SAKAS 581_ QEADN 586_ VLILQ 591_ DRKLV 596_ TGPGK 601_ RYLQV 606_ SKNRF 611_ DGDVG 616_ VFPLE 621_ FNKNS 626_ LTFSI 631_ PPKNK 636_ ARLKK 641_ IKDDT 646_ GPVAK 651_ KPSSG 656_ KKGAT 661_ TQNSE 666_ ICSGQ 671_ APTPD 676_QPDTS
1: Mitochondrial helicase involved in mtDNA replication and repair (PubMed:12975372, PubMed:15167897, PubMed:17324440, PubMed:18039713, PubMed:18971204, PubMed:25824949, PubMed:26887820, PubMed:27226550). Might have a role in mtDNA repair (PubMed:27226550). Has DNA strand separation activity needed to form a processive replication fork for leading strand synthesis which is catalyzed by the formation of a replisome complex with POLG and mtSDB (PubMed:12975372, PubMed:15167897, PubMed:18039713, PubMed:22383523, PubMed:26887820, PubMed:27226550). Preferentially unwinds DNA substrates with pre-existing 5'-and 3'- single-stranded tails but is also active on a 5'- flap substrate (PubMed:12975372, PubMed:15167897, PubMed:18039713, PubMed:22383523, PubMed:26887820, PubMed:27226550). Can dissociate the invading strand of immobile or mobile D-loop DNA structures irrespective of the single strand polarity of the third strand (PubMed:27226550). In addition to its DNA strand separation activity, also has DNA strand annealing, DNA strand-exchange and DNA branch migration activities (PubMed:22383523, PubMed:26887820, PubMed:27226550)
2: Lack DNA unwinding and ATP hydrolysis activities (PubMed:18039713). Does not bind single-stranded or double-stranded DNA (PubMed:18039713)