Gene name: ERCC6

Uniprot entry:

Q03468

Protein names:

DNA excision repair protein ERCC-6 (EC 3.6.4.-) (ATP-dependent helicase ERCC6) (Cockayne syndrome protein CSB)

Protein sequence:

1_MPNEG 6_ IPHSS 11_ QTQEQ 16_ DCLQS 21_ QPVSN 26_ NEEMA 31_ IKQES 36_ GGDGE 41_ VEEYL 46_ SFRSV 51_ GDGLS 56_ TSAVG 61_ CASAA 66_ PRRGP 71_ ALLHI 76_ DRHQI 81_ QAVEP 86_ SAQAL 91_ ELQGL 96_ GVDVY 101_ DQDVL 106_ EQGVL 111_ QQVDN 116_ AIHEA 121_ SRASQ 126_ LVDVE 131_ KEYRS 136_ VLDDL 141_ TSCTT 146_ SLRQI 151_ NKIIE 156_ QLSPQ 161_ AATSR 166_ DINRK 171_ LDSVK 176_ RQKYN 181_ KEQQL 186_ KKITA 191_ KQKHL 196_ QAILG 201_ GAEVK 206_ IELDH 211_ ASLEE 216_ DAEPG 221_ PSSLG 226_ SMLMP 231_ VQETA 236_ WEELI 241_ RTGQM 246_ TPFGT 251_ QIPQK 256_ QEKKP 261_ RKIML 266_ NEASG 271_ FEKYL 276_ ADQAK 281_ LSFER 286_ KKQGC 291_ NKRAA 296_ RKAPA 301_ PVTPP 306_ APVQN 311_ KNKPN 316_ KKARV 321_ LSKKE 326_ ERLKK 331_ HIKKL 336_ QKRAL 341_ QFQGK 346_ VGLPK 351_ ARRPW 356_ ESDMR 361_ PEAEG 366_ DSEGE 371_ ESEYF 376_ PTEEE 381_ EEEED 386_ DEVEG 391_ AEADL 396_ SGDGT 401_ DYELK 406_ PLPKG 411_ GKRQK 416_ KVPVQ 421_ EIDDD 426_ FFPSS 431_ GEEAE 436_ AASVG 441_ EGGGG 446_ GRKVG 451_ RYRDD 456_ GDEDY 461_ YKQRL 466_ RRWNK 471_ LRLQD 476_ KEKRL 481_ KLEDD 486_ SEESD 491_ AEFDE 496_ GFKVP 501_ GFLFK 506_ KLFKY 511_ QQTGV 516_ RWLWE 521_ LHCQQ 526_ AGGIL 531_ GDEMG 536_ LGKTI 541_ QIIAF 546_ LAGLS 551_ YSKIR 556_ TRGSN 561_ YRFEG 566_ LGPTV 571_ IVCPT 576_ TVMHQ 581_ WVKEF 586_ HTWWP 591_ PFRVA 596_ ILHET 601_ GSYTH 606_ KKEKL 611_ IRDVA 616_ HCHGI 621_ LITSY 626_ SYIRL 631_ MQDDI 636_ SRYDW 641_ HYVIL 646_ DEGHK 651_ IRNPN 656_ AAVTL 661_ ACKQF 666_ RTPHR 671_ IILSG 676_ SPMQN 681_ NLREL 686_ WSLFD 691_ FIFPG 696_ KLGTL 701_ PVFME 706_ QFSVP 711_ ITMGG 716_ YSNAS 721_ PVQVK 726_ TAYKC 731_ ACVLR 736_ DTINP 741_ YLLRR 746_ MKSDV 751_ KMSLS 756_ LPDKN 761_ EQVLF 766_ CRLTD 771_ EQHKV 776_ YQNFV 781_ DSKEV 786_ YRILN 791_ GEMQI 796_ FSGLI 801_ ALRKI 806_ CNHPD 811_ LFSGG 816_ PKNLK 821_ GLPDD 826_ ELEED 831_ QFGYW 836_ KRSGK 841_ MIVVE 846_ SLLKI 851_ WHKQG 856_ QRVLL 861_ FSQSR 866_ QMLDI 871_ LEVFL 876_ RAQKY 881_ TYLKM 886_ DGTTT 891_ IASRQ 896_ PLITR 901_ YNEDT 906_ SIFVF 911_ LLTTR 916_ VGGLG 921_ VNLTG 926_ ANRVV 931_ IYDPD 936_ WNPST 941_ DTQAR 946_ ERAWR 951_ IGQKK 956_ QVTVY 961_ RLLTA 966_ GTIEE 971_ KIYHR 976_ QIFKQ 981_ FLTNR 986_ VLKDP 991_ KQRRF 996_ FKSND 1001_ LYELF 1006_ TLTSP 1011_ DASQS 1016_ TETSA 1021_ IFAGT 1026_ GSDVQ 1031_ TPKCH 1036_ LKRRI 1041_ QPAFG 1046_ ADHDV 1051_ PKRKK 1056_ FPASN 1061_ ISVND 1066_ ATSSE 1071_ EKSEA 1076_ KGAEV 1081_ NAVTS 1086_ NRSDP 1091_ LKDDP 1096_ HMSSN 1101_ VTSND 1106_ RLGEE 1111_ TNAVS 1116_ GPEEL 1121_ SVISG 1126_ NGECS 1131_ NSSGT 1136_ GKTSM 1141_ PSGDE 1146_ SIDEK 1151_ LGLSY 1156_ KRERP 1161_ SQAQT 1166_ EAFWE 1171_ NKQME 1176_ NNFYK 1181_ HKSKT 1186_ KHHSV 1191_ AEEET 1196_ LEKHL 1201_ RPKQK 1206_ PKNSK 1211_ HCRDA 1216_ KFEGT 1221_ RIPHL 1226_ VKKRR 1231_ YQKQD 1236_ SENKS 1241_ EAKEQ 1246_ SNDDY 1251_ VLEKL 1256_ FKKSV 1261_ GVHSV 1266_ MKHDA 1271_ IMDGA 1276_ SPDYV 1281_ LVEAE 1286_ ANRVA 1291_ QDALK 1296_ ALRLS 1301_ RQRCL 1306_ GAVSG 1311_ VPTWT 1316_ GHRGI 1321_ SGAPA 1326_ GKKSR 1331_ FGKKR 1336_ NSNFS 1341_ VQHPS 1346_ STSPT 1351_ EKCQD 1356_ GIMKK 1361_ EGKDN 1366_ VPEHF 1371_ SGRAE 1376_ DADSS 1381_ SGPLA 1386_ SSSLL 1391_ AKMRA 1396_ RNHLI 1401_ LPERL 1406_ ESESG 1411_ HLQEA 1416_ SALLP 1421_ TTEHD 1426_ DLLVE 1431_ MRNFI 1436_ AFQAH 1441_ TDGQA 1446_ STREI 1451_ LQEFE 1456_ SKLSA 1461_ SQSCV 1466_ FRELL 1471_ RNLCT 1476_ FHRTS 1481_ GGEGI 1486_WKLKP

Protein annotations

Protein functions:

1: Essential factor involved in transcription-coupled nucleotide excision repair (TC-NER), a process during which RNA polymerase II-blocking lesions are rapidly removed from the transcribed strand of active genes (PubMed:16246722, PubMed:20541997, PubMed:22483866, PubMed:26620705, PubMed:32355176, PubMed:34526721, PubMed:38316879, PubMed:38600235, PubMed:38600236). Plays a central role in the initiation of the TC-NER process: specifically recognizes and binds RNA polymerase II stalled at a lesion, and mediates recruitment of ERCC8/CSA, initiating DNA damage excision by TFIIH recruitment (PubMed:32355176, PubMed:34526721, PubMed:38600235, PubMed:38600236). Upon DNA-binding, it locally modifies DNA conformation by wrapping the DNA around itself, thereby modifying the interface between stalled RNA polymerase II and DNA (PubMed:15548521). Acts as a chromatin remodeler at DSBs; DNA-dependent ATPase-dependent activity is essential for this function (PubMed:16246722, PubMed:9565609). Plays an important role in regulating the choice of the DNA double-strand breaks (DSBs) repair pathway and G2/M checkpoint activation; DNA-dependent ATPase activity is essential for this function (PubMed:25820262). Regulates the DNA repair pathway choice by inhibiting non-homologous end joining (NHEJ), thereby promoting the homologous recombination (HR)-mediated repair of DSBs during the S/G2 phases of the cell cycle (PubMed:25820262). Mediates the activation of the ATM- and CHEK2-dependent DNA damage responses thus preventing premature entry of cells into mitosis following the induction of DNA DSBs (PubMed:25820262). Remodels chromatin by evicting histones from chromatin flanking DSBs, limiting RIF1 accumulation at DSBs thereby promoting BRCA1-mediated HR (PubMed:29203878). Required for stable recruitment of ELOA and CUL5 to DNA damage sites (PubMed:28292928). Also involved in UV-induced translocation of ERCC8 to the nuclear matrix (PubMed:26620705). Essential for neuronal differentiation and neuritogenesis; regulates transcription and chromatin remodeling activities required during neurogenesis (PubMed:24874740)