DNA polymerase subunit gamma-1 (EC 2.7.7.7) (Mitochondrial DNA polymerase catalytic subunit) (PolG-alpha)
1_MSRLL 6_ WRKVA 11_ GATVG 16_ PGPVP 21_ APGRW 26_ VSSSV 31_ PASDP 36_ SDGQR 41_ RRQQQ 46_ QQQQQ 51_ QQQQQ 56_ PQQPQ 61_ VLSSE 66_ GGQLR 71_ HNPLD 76_ IQMLS 81_ RGLHE 86_ QIFGQ 91_ GGEMP 96_ GEAAV 101_ RRSVE 106_ HLQKH 111_ GLWGQ 116_ PAVPL 121_ PDVEL 126_ RLPPL 131_ YGDNL 136_ DQHFR 141_ LLAQK 146_ QSLPY 151_ LEAAN 156_ LLLQA 161_ QLPPK 166_ PPAWA 171_ WAEGW 176_ TRYGP 181_ EGEAV 186_ PVAIP 191_ EERAL 196_ VFDVE 201_ VCLAE 206_ GTCPT 211_ LAVAI 216_ SPSAW 221_ YSWCS 226_ QRLVE 231_ ERYSW 236_ TSQLS 241_ PADLI 246_ PLEVP 251_ TGASS 256_ PTQRD 261_ WQEQL 266_ VVGHN 271_ VSFDR 276_ AHIRE 281_ QYLIQ 286_ GSRMR 291_ FLDTM 296_ SMHMA 301_ ISGLS 306_ SFQRS 311_ LWIAA 316_ KQGKH 321_ KVQPP 326_ TKQGQ 331_ KSQRK 336_ ARRGP 341_ AISSW 346_ DWLDI 351_ SSVNS 356_ LAEVH 361_ RLYVG 366_ GPPLE 371_ KEPRE 376_ LFVKG 381_ TMKDI 386_ RENFQ 391_ DLMQY 396_ CAQDV 401_ WATHE 406_ VFQQQ 411_ LPLFL 416_ ERCPH 421_ PVTLA 426_ GMLEM 431_ GVSYL 436_ PVNQN 441_ WERYL 446_ AEAQG 451_ TYEEL 456_ QREMK 461_ KSLMD 466_ LANDA 471_ CQLLS 476_ GERYK 481_ EDPWL 486_ WDLEW 491_ DLQEF 496_ KQKKA 501_ KKVKK 506_ EPATA 511_ SKLPI 516_ EGAGA 521_ PGDPM 526_ DQEDL 531_ GPCSE 536_ EEEFQ 541_ QDVMA 546_ RACLQ 551_ KLKGT 556_ TELLP 561_ KRPQH 566_ LPGHP 571_ GWYRK 576_ LCPRL 581_ DDPAW 586_ TPGPS 591_ LLSLQ 596_ MRVTP 601_ KLMAL 606_ TWDGF 611_ PLHYS 616_ ERHGW 621_ GYLVP 626_ GRRDN 631_ LAKLP 636_ TGTTL 641_ ESAGV 646_ VCPYR 651_ AIESL 656_ YRKHC 661_ LEQGK 666_ QQLMP 671_ QEAGL 676_ AEEFL 681_ LTDNS 686_ AIWQT 691_ VEELD 696_ YLEVE 701_ AEAKM 706_ ENLRA 711_ AVPGQ 716_ PLALT 721_ ARGGP 726_ KDTQP 731_ SYHHG 736_ NGPYN 741_ DVDIP 746_ GCWFF 751_ KLPHK 756_ DGNSC 761_ NVGSP 766_ FAKDF 771_ LPKME 776_ DGTLQ 781_ AGPGG 786_ ASGPR 791_ ALEIN 796_ KMISF 801_ WRNAH 806_ KRISS 811_ QMVVW 816_ LPRSA 821_ LPRAV 826_ IRHPD 831_ YDEEG 836_ LYGAI 841_ LPQVV 846_ TAGTI 851_ TRRAV 856_ EPTWL 861_ TASNA 866_ RPDRV 871_ GSELK 876_ AMVQA 881_ PPGYT 886_ LVGAD 891_ VDSQE 896_ LWIAA 901_ VLGDA 906_ HFAGM 911_ HGCTA 916_ FGWMT 921_ LQGRK 926_ SRGTD 931_ LHSKT 936_ ATTVG 941_ ISREH 946_ AKIFN 951_ YGRIY 956_ GAGQP 961_ FAERL 966_ LMQFN 971_ HRLTQ 976_ QEAAE 981_ KAQQM 986_ YAATK 991_ GLRWY 996_ RLSDE 1001_ GEWLV 1006_ RELNL 1011_ PVDRT 1016_ EGGWI 1021_ SLQDL 1026_ RKVQR 1031_ ETARK 1036_ SQWKK 1041_ WEVVA 1046_ ERAWK 1051_ GGTES 1056_ EMFNK 1061_ LESIA 1066_ TSDIP 1071_ RTPVL 1076_ GCCIS 1081_ RALEP 1086_ SAVQE 1091_ EFMTS 1096_ RVNWV 1101_ VQSSA 1106_ VDYLH 1111_ LMLVA 1116_ MKWLF 1121_ EEFAI 1126_ DGRFC 1131_ ISIHD 1136_ EVRYL 1141_ VREED 1146_ RYRAA 1151_ LALQI 1156_ TNLLT 1161_ RCMFA 1166_ YKLGL 1171_ NDLPQ 1176_ SVAFF 1181_ SAVDI 1186_ DRCLR 1191_ KEVTM 1196_ DCKTP 1201_ SNPTG 1206_ MERRY 1211_ GIPQG 1216_ EALDI 1221_ YQIIE 1226_ LTKGS 1231_LEKRS
1: Catalytic subunit of DNA polymerase gamma solely responsible for replication of mitochondrial DNA (mtDNA). Replicates both heavy and light strands of the circular mtDNA genome using a single-stranded DNA template, RNA primers and the four deoxyribonucleoside triphosphates as substrates (PubMed:11477093, PubMed:11897778, PubMed:15917273, PubMed:19837034, PubMed:9558343). Has 5' -> 3' polymerase activity. Functionally interacts with TWNK and SSBP1 at the replication fork to form a highly processive replisome, where TWNK unwinds the double-stranded DNA template prior to replication and SSBP1 covers the parental heavy strand to enable continuous replication of the entire mitochondrial genome. A single nucleotide incorporation cycle includes binding of the incoming nucleotide at the insertion site, a phosphodiester bond formation reaction that extends the 3'-end of the primer DNA, and translocation of the primer terminus to the post-insertion site. After completing replication of a mtDNA strand, mediates 3' -> 5' exonucleolytic degradation at the nick to enable proper ligation (PubMed:11477093, PubMed:11897778, PubMed:15167897, PubMed:15917273, PubMed:19837034, PubMed:26095671, PubMed:9558343). Highly accurate due to high nucleotide selectivity and 3' -> 5' exonucleolytic proofreading. Proficiently corrects base substitutions, single-base additions and deletions in non-repetitive sequences and short repeats, but displays lower proofreading activity when replicating longer homopolymeric stretches. Exerts exonuclease activity toward single-stranded DNA and double-stranded DNA containing 3'-terminal mispairs. When a misincorporation occurs, transitions from replication to a pro-nucleolytic editing mode and removes the missincorporated nucleoside in the exonuclease active site. Proceeds via an SN2 nucleolytic mechanism in which Asp-198 catalyzes phosphodiester bond hydrolysis and Glu-200 stabilizes the leaving group. As a result the primer strand becomes one nucleotide shorter and is positioned in the post-insertion site, ready to resume DNA synthesis (PubMed:10827171, PubMed:11477094, PubMed:11504725, PubMed:37202477). Exerts 5'-deoxyribose phosphate (dRP) lyase activity and mediates repair-associated mtDNA synthesis (gap filling) in base-excision repair pathway. Catalyzes the release of the 5'-terminal 2-deoxyribose-5-phosphate sugar moiety from incised apurinic/apyrimidinic (AP) sites to produce a substrate for DNA ligase. The dRP lyase reaction does not require divalent metal ions and likely proceeds via a Schiff base intermediate in a beta-elimination reaction mechanism (PubMed:9770471)