Protein: DNA excision repair protein ERCC-6 (EC 3.6.4.-) (ATP-dependent helicase ERCC6) (Cockayne syndrome protein CSB) (F8VPZ5)
Organism: Mus musculus (Mouse)
Function: Essential factor involved in transcription-coupled nucleotide excision repair which allows RNA polymerase II-blocking lesions to be rapidly removed from the transcribed strand of active genes (By similarity). 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 (By similarity). It is required for transcription-coupled repair complex formation. It recruits the CSA complex (DCX(ERCC8) complex), nucleotide excision repair proteins and EP300 to the sites of RNA polymerase II-blocking lesions (By similarity). 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 (By similarity). 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 (By similarity). Mediates the activation of the ATM- and CHEK2-dependent DNA damage responses thus preventing the premature exit from the G2/M checkpoint (By similarity). Acts as a chromatin remodeler at DSBs; DNA-dependent ATPase-dependent activity is essential for this function (By similarity). Remodels chromatin by evicting histones from chromatin flanking DSBs, limiting RIF1 accumulation at DSBs thereby promoting BRCA1-mediated HR (By similarity). Required for stable recruitment of ELOA and CUL5 to DNA damage sites (By similarity). Involved in UV-induced translocation of ERCC8 to the nuclear matrix (By similarity). Essential for neuronal differentiation and neuritogenesis; regulates transcription and chromatin remodeling activities required during neurogenesis (By similarity).
Subcellular location: Nucleus {ECO:0000250|UniProtKB:Q03468}. Chromosome {ECO:0000250|UniProtKB:Q03468}. Note=Recognizes and binds RNA polymerase II stalled at DNA damage sites. {ECO:0000250|UniProtKB:Q03468}.
Interaction partners:
- DNA excision repair protein ERCC-8
- DNA-directed RNA polymerase II subunit RPB1
- DNA excision repair protein ERCC-5
- DNA-directed RNA polymerase II subunit RPB2
- DNA-directed RNA polymerase II subunit RPB9
- DNA repair protein complementing XP-A cells homolog
- UV-stimulated scaffold protein A
- DNA damage-binding protein 1
- DNA-directed RNA polymerases I, II, and III subunit RPABC2
- DNA-directed RNA polymerase II subunit RPB4
Sequence:
MFHEEVPNSTHPQEQDCLPSQHANAYKDMPVGQENGGVSEAGECLSSTSCEYGPSTSAEACVLAATRRGPTLLHIDRHQIPAVEPSAQALELQGLGVDVYDQAVLEQGVLQQVDSAMHEASCVAQLADAEKEYQSVLDDLMSCTTSLRQINKIIEQLSPQAASNRDINRKLDSVKRQKYNKEQQLKKITAKQKRLQAILGGAGVQVELDHASLEEDDAEPGPSCLGSMLMPAQETAWEELIRTGQMTPFGTPAPQKQEKKPRKIMLNEASGFEKYLAEQAQLSFERKKQAATKRTAKKAIVISESSRAAIETKADQRSQVLSQTDKRLKKHSRKLQRRALQFQGKVGLPSGKKPLEPEVRPEAEGDTEGEESGSSPTDGEEEEEQEEEEGVASLSSDDVSYELKPLRKRQKYQKKVPVQEIDDDFFPSSEEEDEAMEGRGGGRKVARRQDDGDEDYYKQRLRRWNRLRLQDKEKRLKLEDDSEESDAEFDEGFKVPGFLFKKLFKYQQTGVRWLWELHCQQAGGILGDEMGLGKTIQIIAFLAGLSYSKIRTRGSNYRFEGLGPTIIVCPTTVMHQWVKEFHTWWPPFRVAVLHETGSYTHKKERLIRDIVYCHGVLITSYSYIRLMQDDISRHDWHYVILDEGHKIRNPNAAVTLACKQFRTPHRIILSGSPMQNNLRELWSLFDFIFPGKLGTLPVFMEQFSVPITMGGYSNASPVQVKTAYKCACVLRDTINPYLLRRMKSDVKMSLSLPDKNEQVLFCRLTDEQHKVYQNFIDSKAVYRILNGENQIFSGLVALRKICNHPDLFSGGPKNASGPPEDELEEEQFGHWRRSGKMIVVESLLKIWHRQGQRVLLFSQSRQMLHILEVFLRAHKYSYLKMDGTTTIASRQPLITKYNEDTSIFVFLLTTRVGGLGVNLTGANRVIIYDPDWNPSTDTQARERAWRIGQKKQVTVYRLLTAGTIEEKIYHRQIFKQFLTNRVLKDPKQRRFFKSNDLYELFTLTSPDASQGTETSAIFAGTGSSIQTPKCQLKKRTSTVLGTDPKCKKPPVSDTPANAATLIGEKPKAAGATGRSVTSGESGPFKGDHDTNGNRASSVAFGEETDAGSTLEHLSVMSGDGKHSDSPTVDHTSRPPVEASTSEKQGSSYAGARCQAQTEPVPMSEQMEGQFSKYKSKRKHDASEEETTEKRPQPKQKAKNSKHCRDAKFEGTRVPHLVKKRRYRQQTSEQEGGAKDRSSDDYVLEKLFKKSVGVHSVVRHDAIIDGSSPDYVLVEAEANRVAQDALKALRLSRQQCLGAASGVPTWTGHRGISGAPTGVKNRFGQKRDSSLPVQHPSSLTEKTQNNMKKEGKAHTPEHFSGKEDGASVSGAPSSSSLLARMRARNHMILPERLESDSEHLAEAAAVPPCGTEHDDLLVDMRNFIAFQAQVDGQASTQEILQEFESKLSVAQSCVFRELLRNLCNFHRTPGGEGIWKLKPEYC