ERCC6
UniProt ID: Q03468
Entrez ID: 2074
Protein name: DNA excision repair protein ERCC-6 (EC 3.6.4.-) (ATP-dependent helicase ERCC6) (Cockayne syndrome protein CSB)
External links: UniprotKB HGNC RefSeq COSMIC
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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 (PubMed:20541997, PubMed:26620705, PubMed:16246722). Upon DNA-binding, it locally modifies DNA con...
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 (PubMed:20541997, PubMed:26620705, PubMed:16246722). 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). It is required for transcription-coupled repair complex formation (PubMed:16916636). It recruits the CSA complex (DCX(ERCC8) complex), nucleotide excision repair proteins and EP300 to the sites of RNA polymerase II-blocking lesions (PubMed:16916636). 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). Acts as a chromatin remodeler at DSBs; DNA-dependent ATPase-dependent activity is essential for this function. 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). 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). {ECO:0000269|PubMed:15548521, ECO:0000269|PubMed:16246722, ECO:0000269|PubMed:16916636, ECO:0000269|PubMed:20541997, ECO:0000269|PubMed:22483866, ECO:0000269|PubMed:24874740, ECO:0000269|PubMed:25820262, ECO:0000269|PubMed:26620705, ECO:0000269|PubMed:28292928, ECO:0000269|PubMed:29203878}.
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GO - Biological processes (BP):
base-excision repair [GO:0006284]; chromatin remodeling [GO:0006338]; DNA damage checkpoint signaling [GO:0000077]; DNA protection [GO:0042262]; DNA repair [GO:0006281]; double-strand break repair via classical nonhomologous end joining [GO:0097680]; intrinsic apoptotic signaling pathway in response...
base-excision repair [GO:0006284]; chromatin remodeling [GO:0006338]; DNA damage checkpoint signaling [GO:0000077]; DNA protection [GO:0042262]; DNA repair [GO:0006281]; double-strand break repair via classical nonhomologous end joining [GO:0097680]; intrinsic apoptotic signaling pathway in response to DNA damage [GO:0008630]; JNK cascade [GO:0007254]; multicellular organism growth [GO:0035264]; negative regulation of double-strand break repair via nonhomologous end joining [GO:2001033]; neurogenesis [GO:0022008]; neuron differentiation [GO:0030182]; neuron projection development [GO:0031175]; photoreceptor cell maintenance [GO:0045494]; positive regulation of DNA repair [GO:0045739]; positive regulation of DNA-templated transcription, elongation [GO:0032786]; positive regulation of double-strand break repair via homologous recombination [GO:1905168]; positive regulation of histone acetylation [GO:0035066]; positive regulation of transcription by RNA polymerase I [GO:0045943]; positive regulation of transcription by RNA polymerase II [GO:0045944]; positive regulation of transcription by RNA polymerase III [GO:0045945]; positive regulation of transcription initiation from RNA polymerase II promoter [GO:0060261]; pyrimidine dimer repair [GO:0006290]; regulation of DNA-templated transcription, elongation [GO:0032784]; regulation of transcription elongation from RNA polymerase II promoter [GO:0034243]; response to gamma radiation [GO:0010332]; response to oxidative stress [GO:0006979]; response to superoxide [GO:0000303]; response to toxic substance [GO:0009636]; response to UV [GO:0009411]; response to UV-B [GO:0010224]; response to X-ray [GO:0010165]; single strand break repair [GO:0000012]; transcription by RNA polymerase II [GO:0006366]; transcription-coupled nucleotide-excision repair [GO:0006283]; transcription elongation from RNA polymerase I promoter [GO:0006362]
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GO - Molecular function (MF):
ATP binding [GO:0005524]; ATP-dependent activity, acting on DNA [GO:0008094]; ATP-dependent chromatin remodeler activity [GO:0140658]; ATP hydrolysis activity [GO:0016887]; chromatin binding [GO:0003682]; DNA binding [GO:0003677]; helicase activity [GO:0004386]; protein-containing complex binding [G...
ATP binding [GO:0005524]; ATP-dependent activity, acting on DNA [GO:0008094]; ATP-dependent chromatin remodeler activity [GO:0140658]; ATP hydrolysis activity [GO:0016887]; chromatin binding [GO:0003682]; DNA binding [GO:0003677]; helicase activity [GO:0004386]; protein-containing complex binding [GO:0044877]; protein C-terminus binding [GO:0008022]; protein N-terminus binding [GO:0047485]; protein tyrosine kinase activator activity [GO:0030296]; RNA polymerase binding [GO:0070063]
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GO - Cellular component (CC):
B-WICH complex [GO:0110016]; nucleolus [GO:0005730]; nucleoplasm [GO:0005654]; nucleus [GO:0005634]; site of DNA damage [GO:0090734]; transcription elongation factor complex [GO:0008023]...
B-WICH complex [GO:0110016]; nucleolus [GO:0005730]; nucleoplasm [GO:0005654]; nucleus [GO:0005634]; site of DNA damage [GO:0090734]; transcription elongation factor complex [GO:0008023]
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ERCC6
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*NOTE: the Kaplan plots were collected from OncoLnc