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Showing ERCC6 — RAD26 is a alias.

ERCC6

DNA excision repair protein ERCC-6 · UniProt Q03468

Length
1493 aa
Mass
168.4 kDa
Annotated
2026-06-09
100 papers in source corpus 26 papers cited in narrative 26 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 8/8 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

ERCC6/CSB is the central transcription-repair coupling factor for transcription-coupled nucleotide excision repair (TC-NER), the pathway that preferentially removes lesions from the transcribed strand of active genes (PMID:1339317, PMID:7957102). It is a DNA-stimulated ATPase of the helicase-motif family that nonetheless lacks helicase activity and does not by itself dissociate stalled RNA polymerase II; rather, it binds DNA and elongating RNAPII ternary complexes in a manner that strictly requires ATP hydrolysis, and physically contacts core NER factors including XPA, TFIIH, and TFIIE-p34 (PMID:1339317, PMID:8999876, PMID:9372911). CSB/Rad26 recognizes RNAPII arrested at lesions through a common stalling-recognition mechanism augmented by additional lesion-specific contacts, and cooperates with Elf1/ELOF1 at the initial lesion-recognition step (PMID:38194460); its ATPase module allosterically remodels DNA and Pol II to assist progression past less bulky obstacles (PMID:34853308). CSB function in TC-NER is gated by the elongation factor Spt5/Spt4: Spt5 (with its Bur-kinase-phosphorylated C-terminal repeat) assembles a Pol II-associated complex that suppresses Rad26-independent repair, and deletion of SPT4 restores TC-NER genome-wide in CSB-deficient cells, particularly downstream of the +1 nucleosome where TFIIH occupancy is low (PMID:11101522, PMID:20042611, PMID:32690696). When lesions cannot be rapidly removed, CSB/Rad26 partners with Def1 in chromatin to coordinate ubiquitination and proteolysis of stalled RNAPII and its eviction from damaged loci (PMID:11859374, PMID:23991048). CSB activity is regulated post-translationally—directly phosphorylated by the Mec1/ATR checkpoint kinase to promote TC-NER (PMID:19901073) and stabilized after UV damage by the UVSSA–USP7 complex (PMID:22466612). Beyond canonical NER, CSB promotes error-free transcriptional bypass of UV photoproducts and alkylated bases and constitutively evicts Spt5 from chromatin, defining a repair-independent elongation function (PMID:12024048, PMID:30790631). Loss-of-function mutations in ERCC6 cause Cockayne syndrome, a connection interpretable in the context of its DNA-remodeling structural mechanism (PMID:34853308).

Mechanistic history

Synthesis pass · year-by-year structured walk · 14 steps
  1. 1992 High

    Established the gene's identity and core biological assignment—a helicase-motif protein specifically required for transcription-coupled, not global, nucleotide excision repair.

    Evidence Molecular cloning and complementation of CS-B cells with mutation analysis

    PMID:1339317

    Open questions at the time
    • Did not establish biochemical activity of the protein
    • Helicase motifs implied but enzymatic function untested
  2. 1994 High

    Demonstrated functional conservation by showing the yeast ortholog RAD26 carries out strand-specific TCR, enabling genetic dissection in a tractable system.

    Evidence Gene disruption and strand-specific CPD repair assay in S. cerevisiae

    PMID:7957102

    Open questions at the time
    • Mechanism of strand selectivity unresolved
    • No biochemical activity defined
  3. 1997 High

    Defined the protein as a DNA-stimulated ATPase that is not a helicase and does not displace Pol II, and showed ATP-hydrolysis-dependent engagement of RNAPII ternary complexes plus contacts with core NER factors.

    Evidence Recombinant protein ATPase/helicase assays, ternary-complex pulldowns with ATPase mutants, Co-IP with XPA/TFIIH/TFIIE-p34 (building on yeast Rad26 ATPase work)

    PMID:8702468 PMID:8999876 PMID:9372911

    Open questions at the time
    • How ATPase activity translates into repair coupling not defined
    • Structural basis of Pol II engagement unknown at this stage
  4. 2001 Medium

    Reframed CSB as a transcription elongation factor whose absence impairs RNAPII elongation in vivo, linking the elongation defect to Cockayne syndrome developmental phenotypes.

    Evidence In vivo galactose-inducible transcription and growth assays in rad26 yeast (with prior cell-extract transcription/TFIIH-mobilization data)

    PMID:11713297 PMID:9566886 PMID:9880486

    Open questions at the time
    • Direct elongation mechanism not biochemically reconstituted
    • Relationship between elongation role and TCR role unresolved
  5. 2002 High

    Identified the Def1 partnership coupling TCR to RNAPII degradation, explaining how persistently stalled polymerases are cleared when repair cannot proceed.

    Evidence Co-IP, chromatin fractionation, genetic epistasis, RNAPII ubiquitination/degradation assays

    PMID:11859374

    Open questions at the time
    • Ubiquitin ligase machinery details not fully defined
    • Trigger choosing repair versus degradation unknown
  6. 2002 Medium

    Distinguished a repair-independent function: CSB/Rad26 promotes transcription through alkylated bases separately from NER/BER, broadening its role beyond lesion excision.

    Evidence MMS sensitivity and synergistic mutant analysis with transcription readout in yeast

    PMID:12024048

    Open questions at the time
    • Biochemical basis of damaged-base bypass unresolved
    • Generality across lesion types untested
  7. 2009 Medium

    Revealed CSB activity is post-translationally activated by the apical checkpoint kinase Mec1/ATR, connecting DNA-damage signaling directly to TC-NER kinetics.

    Evidence In vivo phosphorylation, phosphosite mutant TC-NER assay, kinase mutant epistasis

    PMID:19901073

    Open questions at the time
    • Structural/functional consequence of phosphorylation undefined
    • Single lab; human CSB phosphoregulation not directly demonstrated here
  8. 2009 Medium

    Mapped the Spt5/Spt4 suppression mechanism that gates Rad26-independent repair, identifying the Bur-phosphorylated Spt5 CTR as a Pol II-associated suppressor platform.

    Evidence Genetic epistasis with spt4/spt5-CTR/bur mutants, in vivo TCR assays, Co-IP (with prior spt4 suppressor genetics)

    PMID:11101522 PMID:20042611

    Open questions at the time
    • Direct competition between CSB and the suppressor complex not biochemically resolved
  9. 2012 High

    Identified UVSSA-USP7 as the complex stabilizing CSB after UV, defining how CSB protein levels and Pol II phosphostate are maintained during repair.

    Evidence Complementation cloning, Co-IP, CSB stability Westerns, recovery-of-RNA-synthesis assay in human cells

    PMID:22466612

    Open questions at the time
    • Mechanism by which USP7 deubiquitinates CSB versus Pol II not fully separated
  10. 2016 Medium

    Refined the TCR hierarchy by showing senataxin/Sen1 acts more universally than Rad26, indicating CSB covers a major but incomplete subset of transcribed-strand lesions.

    Evidence Genetic deletion epistasis (sen1/rad26/spt4) with strand-specific CPD repair (with parallel ChIP showing CSB-dependent Pol II removal)

    PMID:23991048 PMID:27179024

    Open questions at the time
    • Functional interplay between Sen1 and Rad26 at shared sites unresolved
  11. 2020 High

    Resolved where in genes CSB matters: its ATPase is critical for TC-NER downstream of the +1 nucleosome where TFIIH is low and Spt4/Spt5 is high, with SPT4 loss rescuing repair genome-wide.

    Evidence Single-nucleotide CPD-seq, chromatin occupancy analysis, rad26/spt4 epistasis

    PMID:32690696

    Open questions at the time
    • Mechanistic link between chromatin context and CSB requirement not fully defined
  12. 2021 Medium

    Provided a structural/dynamic model in which the CSB ATPase module allosterically remodels DNA and Pol II, offering a framework to interpret Cockayne syndrome mutations.

    Evidence Cryo-EM modeling, molecular dynamics, community analysis

    PMID:34853308

    Open questions at the time
    • No experimental mutagenesis validation reported
    • Predicted allosteric pathway not biochemically tested
  13. 2024 High

    Established the lesion-recognition step structurally, showing CSB/Rad26 uses a common Pol II-stalling recognition mode with lesion-specific contacts and cooperates with Elf1/ELOF1.

    Evidence Cryo-EM of Pol II-Rad26 complexes with biochemical and genetic validation

    PMID:38194460

    Open questions at the time
    • Subsequent handoff to NER incision machinery not structurally resolved
    • Human ELOF1-CSB complex not directly determined here
  14. 2024 Medium

    Uncovered a non-canonical role in RNA processing whereby ERCC6-HNRNPM modulates BAX splicing and apoptosis, implicating CSB in PI3K/AKT signaling and cell survival.

    Evidence CRISPR screen, shRNA knockdown, Co-IP, RNA-seq splicing analysis, xenograft

    PMID:40476445

    Open questions at the time
    • Directness of splicing regulation versus indirect effect unresolved
    • Relationship to repair/elongation functions unknown

Open questions

Synthesis pass · forward-looking unresolved questions
  • How CSB hands off the lesion-arrested polymerase to the downstream NER incision machinery, and how its repair, elongation, and RNA-processing functions are coordinated in human cells, remains unresolved.
  • No structural model of the CSB-to-TFIIH/XPA handoff
  • Integration of repair-independent functions with TC-NER undefined
  • Human in vivo phosphoregulation and Def1-equivalent coupling not directly demonstrated

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0140657 ATP-dependent activity 4 GO:0016787 hydrolase activity 2 GO:0003677 DNA binding 1 GO:0140096 catalytic activity, acting on a protein 1
Localization
GO:0000228 nuclear chromosome 3 GO:0005634 nucleus 2
Pathway
R-HSA-73894 DNA Repair 4 R-HSA-74160 Gene expression (Transcription) 3 R-HSA-8953854 Metabolism of RNA 1
Partners
DEF1ELOF1/ELF1HNRNPMRNA POLYMERASE IITFIIE (P34/GTF2E2)TFIIHUVSSAXPA
Complex memberships
RNA polymerase II elongation complexRad26-Def1 complex

Evidence

Reading pass · 26 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1992 ERCC6 encodes a 1493 amino acid protein containing seven consecutive domains conserved between DNA and RNA helicases, and is specifically required for preferential (transcription-coupled) nucleotide excision repair of the transcribed strand of active genes; mutation analysis showed the gene is not essential for cell viability. Molecular cloning, complementation assay in CS-B cells, mutation analysis Cell High 1339317
1993 ERCC6 spans ~82–90 kb genomic region with at least 21 exons; seven helicase signature motifs are each encoded on separate exons; expression produces two mRNA species (5 and 7 kb) via alternative polyadenylation. Genomic cloning, exon mapping, Northern blot, cDNA analysis Nucleic acids research Medium 8382798
1994 RAD26, the S. cerevisiae homolog of ERCC6/CSB, is required for transcription-coupled repair (preferential removal of UV-induced cyclobutane pyrimidine dimers from the transcribed strand of the active RBP2 gene); rad26 disruption does not cause UV sensitivity, indicating TCR is less critical for survival in yeast. Gene disruption, UV survival assay, strand-specific CPD repair assay The EMBO journal High 7957102
1996 Rad26 (yeast ERCC6/CSB ortholog) is a DNA-dependent ATPase; in contrast to E. coli Mfd (weak, DNA-independent ATPase), Rad26 has strict dependence on DNA for ATPase activity. Protein purification from yeast, in vitro ATPase assay The Journal of biological chemistry High 8702468
1997 Human CSB/ERCC6 is a DNA-stimulated ATPase but is NOT a helicase and does NOT dissociate stalled RNA polymerase II ternary complexes; CSB binds DNA and interacts with XPA, TFIIH, and the p34 subunit of TFIIE. Baculovirus overexpression, protein purification, in vitro ATPase assay, helicase assay, Co-IP/pulldown with XPA, TFIIH, TFIIE-p34 The Journal of biological chemistry High 8999876
1997 CSB/ERCC6 interacts with RNA polymerase II engaged in ternary transcription complexes (containing DNA and nascent RNA); this interaction requires hydrolysis of the ATP β-γ phosphoanhydride bond by CSB; CSA does not directly bind Pol II. Biochemical pulldown using oligo(dC)-tailed DNA template, ATPase mutant analysis Molecular and cellular biology High 9372911
1998 RAD26 is required for transcription in yeast cell extracts when nucleotide excision repair is active; in the presence of NER, TFIIH is preferentially mobilized for repair, inhibiting transcription, and RAD26 is required for this inhibition. Cell-free transcription and NER assay with yeast extracts, complementation with purified holo-TFIIH Molecular and cellular biology Medium 9566886
1999 In rad26 mutant yeast cells, loss of TCR is not due to a transcription deficiency but to failure to overcome the nucleotide excision repair block caused by RNA polymerase II stalled at DNA damage sites; repair of transcribed sequences between nucleosomal cores is less efficient in rad26 mutants, pointing to a repair impediment from trapped RNA polymerase. Single-nucleotide resolution CPD repair mapping in vivo, transcription analysis The Journal of biological chemistry Medium 9880486
2000 Deletion of SPT4 (encoding a transcription elongation factor) suppresses the rad26 TCR defect, activating Rad26-independent TCR; this indicates Rad26 functions as an elongation factor rendering transcription TCR-competent, and its requirement is modulated by Spt4. Genome-wide mutagenesis, UV survival, strand-specific repair assay in spt4 rad26 double mutants The EMBO journal High 11101522
2001 RAD26 is required for transcription elongation by RNA polymerase II in vivo; cells lacking RAD26 show reduced transcription of galactose-inducible genes under conditions requiring rapid mRNA synthesis, with considerable growth reduction; this implicates CSB in transcription elongation and suggests impaired elongation underlies developmental defects in Cockayne syndrome. In vivo transcription assay (galactose-inducible gene expression), growth assay in S. cerevisiae Molecular and cellular biology Medium 11713297
2002 Rad26 forms a complex with Def1 in chromatin; Def1 is required for ubiquitination and proteolysis of RNA polymerase II (RNAPII) in response to DNA damage; Rad26-Def1 complex coordinates TCR-mediated DNA repair with RNAPII degradation when lesions cannot be rapidly removed. Co-IP, chromatin fractionation, genetic epistasis, RNAPII ubiquitination and degradation assays Nature High 11859374
2002 RAD26 promotes survival of MMS-treated yeast cells independently of nucleotide excision repair and base excision repair; RAD26 facilitates transcription by RNA polymerase II through MMS-damaged (alkylated) bases, representing a distinct function from its TCR role. MMS sensitivity assay, synergistic mutant analysis (rad26Δ mag1Δ rad14Δ), galactose-inducible gene transcription assay Molecular and cellular biology Medium 12024048
2009 The C-terminal repeat (CTR) domain of Spt5, phosphorylated by Bur kinase, suppresses Rad26-independent TCR by serving as a platform for assembly of a multi-protein suppressor complex associated with Pol II; Spt4 indirectly suppresses Rad26-independent TCR by stabilizing Spt5-Pol II interaction. Genetic epistasis (spt4Δ, spt5 CTR deletion, bur kinase mutants), in vivo TCR assay, Co-IP The Journal of biological chemistry Medium 20042611
2009 Mec1 kinase directly phosphorylates Rad26 (yeast CSB ortholog) in a DNA-damage-dependent manner independently of downstream kinases Rad53, Chk1, Tel1, and Dun1; mutation of the Rad26 phosphorylation site decreases the rate of TC-NER, establishing Mec1-dependent phosphorylation as a direct activation mechanism for TC-NER. In vivo phosphorylation assay, TC-NER strand-specific repair assay in phosphorylation-site mutants, kinase mutant epistasis Molecular and cellular biology Medium 19901073
2012 UVSSA protein forms a complex with USP7 and stabilizes ERCC6/CSB after UV irradiation; loss of UVSSA destabilizes ERCC6 and disrupts TCR; UVSSA restores the hypophosphorylated form of RNA polymerase II after UV irradiation. Complementation cloning, Co-IP, Western blot for ERCC6 stability, RRS (recovery of RNA synthesis) assay Nature genetics High 22466612
2019 At normal or moderately elevated levels, Rad26 promotes error-free transcriptional bypass of UV photoproducts and constitutively evicts Spt5 from chromatin; at very high overexpression (~1/3 of RNAPII levels), Rad26 loses these functions; substantial AMP (but no other nucleotides) is misincorporated opposite UV photoproducts, but Rad26 does not affect this misincorporation. Transcription elongation assay in vivo, ChIP for Spt5 eviction, nucleotide misincorporation mapping Journal of molecular biology Medium 30790631
2020 Rad26 and its ATPase activity are critical for TC-NER downstream of the first (+1) nucleosome in gene coding regions; TC-NER at the TSS-proximal half of the +1 nucleosome is largely Rad26-independent due to high TFIIH occupancy; downstream, the combination of low TFIIH and high Spt4/Spt5 occupancy suppresses TC-NER in Rad26-deficient cells; SPT4 deletion restores TC-NER genome-wide in rad26Δ cells. Single-nucleotide resolution UV damage mapping (CPD-seq), chromatin occupancy analysis, genetic deletion epistasis (rad26Δ, spt4Δ) Proceedings of the National Academy of Sciences of the United States of America High 32690696
2021 Rad26 (CSB ortholog) remodels DNA through an allosteric pathway coupling ATPase module motions to changes in RNA polymerase II and DNA; the mechanism allows CSB-assisted progression past less bulky lesions; Cockayne syndrome disease mutations can be functionally interpreted in this structural context. Cryo-EM structural modeling, molecular dynamics simulation, graph-theoretical community analysis Nature communications Medium 34853308
2021 Rad26 and TFIIS can stimulate bypass of non-template strand (NTS) CAG slip-out-induced transcriptional arrest but NOT template strand (TS) slip-out-induced distal pausing; TS slip-out induces R-loop formation at distal pausing sites; both NTS and TS slip-outs cause Pol II backtracking at proximal sites. In vitro reconstituted yeast transcription system, R-loop mapping, Pol II backtracking assay Nucleic acids research Medium 34197619
2024 Cryo-EM structures of Pol II-Rad26 complexes stalled at different obstacles reveal that Rad26 uses a common mechanism to recognize stalled Pol II, with additional interactions when Pol II is arrested specifically at a DNA lesion; Elf1 (ELOF1 ortholog) induces further interactions between Rad26 and lesion-arrested Pol II; Elf1 and Rad26 cooperate at the initial lesion-recognition step of TC-NER. Cryo-EM structure determination, biochemical pulldown/interaction assays, genetic complementation Proceedings of the National Academy of Sciences of the United States of America High 38194460
2024 ERCC6 interacts with HNRNPM and influences the PI3K/AKT signaling pathway and alternative splicing of BAX pre-mRNA; ERCC6 knockdown increases expression of full-length BAX by reducing exon 2 skipping, promoting apoptosis; exon 2 skipping introduces a premature stop codon in the BH3 domain of BAX. CRISPR screening, shRNA knockdown, Co-IP (ERCC6-HNRNPM interaction), RNA-seq (alternative splicing), xenograft model Advanced science (Weinheim, Baden-Wurttemberg, Germany) Medium 40476445
2013 Rad26 is required for removal of stalled RNA polymerase II from chromatin following UV irradiation; in RAD26-deleted cells, no significant reduction in RNAPII occupancy occurs at damaged active loci (RPB2, PYK1, RPL2B), and transcription of RPB2 is adversely affected during DNA damage repair. Chromatin immunoprecipitation (ChIP) for RNAPII occupancy, UV survival, RT-PCR for transcription PloS one Medium 23991048
2016 Sen1 (senataxin ortholog) plays a more direct role than Rad26 in TCR: unlike Rad26, Sen1 is still required for efficient TCR in cells lacking Spt4, and Sen1 is required for repair at essentially all damaged sites in the transcribed strand, whereas Rad26 is important at many but not all sites. Genetic deletion epistasis (sen1, rad26, spt4 mutants), strand-specific CPD repair assay Nucleic acids research Medium 27179024
2021 CSB (encoded by ERCC6) interacts with VCP (valosin-containing protein) in lens epithelial cells; let-7c-5p-mediated downregulation of ERCC6 disrupts autophagic flux by preventing autophagosomes from combining with lysosomes. Co-immunoprecipitation (CSB-VCP interaction), siRNA knockdown, Western blot, immunofluorescence (LC3B puncta), miRNA mimic transfection Current eye research Low 33703976
2006 A SNP in the ERCC6 5' flanking region (C-6530>G) alters the binding pattern of nuclear proteins (shifts Sp1 binding on C allele to SP1/GATA-1/OCT-1 on G allele) and increases luciferase reporter expression from the G allele; the G allele is associated with higher ERCC6 expression in lymphocytes. EMSA, luciferase reporter assay, CHIP assay Proceedings of the National Academy of Sciences of the United States of America Medium 16754848
2016 UVB-induced repression of ERCC6 in lens epithelial cells is mediated by coordinated hypermethylation of a CpG site at position -441 in the Sp1-binding region of the ERCC6 promoter and histone H3K9 deacetylation, with increased association of DNMT3b and HDAC1 at this site. Bisulfite sequencing (methylation), ChIP for H3K9 acetylation/DNMT3b/HDAC1, RT-PCR and Western blot for ERCC6 expression, apoptosis assay Clinical epigenetics Medium 27231489

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2021 Safety and efficacy of an rAd26 and rAd5 vector-based heterologous prime-boost COVID-19 vaccine: an interim analysis of a randomised controlled phase 3 trial in Russia. Lancet (London, England) 1124 33545094
2020 Safety and immunogenicity of an rAd26 and rAd5 vector-based heterologous prime-boost COVID-19 vaccine in two formulations: two open, non-randomised phase 1/2 studies from Russia. Lancet (London, England) 687 32896291
1992 ERCC6, a member of a subfamily of putative helicases, is involved in Cockayne's syndrome and preferential repair of active genes. Cell 657 1339317
1994 RAD26, the functional S. cerevisiae homolog of the Cockayne syndrome B gene ERCC6. The EMBO journal 229 7957102
1997 Human transcription-repair coupling factor CSB/ERCC6 is a DNA-stimulated ATPase but is not a helicase and does not disrupt the ternary transcription complex of stalled RNA polymerase II. The Journal of biological chemistry 220 8999876
2000 The checkpoint protein Ddc2, functionally related to S. pombe Rad26, interacts with Mec1 and is regulated by Mec1-dependent phosphorylation in budding yeast. Genes & development 199 10950868
2012 Mutations in UVSSA cause UV-sensitive syndrome and destabilize ERCC6 in transcription-coupled DNA repair. Nature genetics 191 22466612
2010 Mutation update for the CSB/ERCC6 and CSA/ERCC8 genes involved in Cockayne syndrome. Human mutation 188 19894250
2002 A Rad26-Def1 complex coordinates repair and RNA pol II proteolysis in response to DNA damage. Nature 188 11859374
1997 Recruitment of the putative transcription-repair coupling factor CSB/ERCC6 to RNA polymerase II elongation complexes. Molecular and cellular biology 167 9372911
1999 A Rad3-Rad26 complex responds to DNA damage independently of other checkpoint proteins. Nature cell biology 162 10559981
1998 Molecular analysis of mutations in the CSB (ERCC6) gene in patients with Cockayne syndrome. American journal of human genetics 127 9443879
1990 Molecular cloning of the human DNA excision repair gene ERCC-6. Molecular and cellular biology 117 2172786
2006 Synergic effect of polymorphisms in ERCC6 5' flanking region and complement factor H on age-related macular degeneration predisposition. Proceedings of the National Academy of Sciences of the United States of America 89 16754848
2009 Laryngeal cancer risk associated with smoking and alcohol consumption is modified by genetic polymorphisms in ERCC5, ERCC6 and RAD23B but not by polymorphisms in five other nucleotide excision repair genes. International journal of cancer 86 19444904
1996 RAD26, the yeast homolog of human Cockayne's syndrome group B gene, encodes a DNA-dependent ATPase. The Journal of biological chemistry 64 8702468
2000 Spt4 modulates Rad26 requirement in transcription-coupled nucleotide excision repair. The EMBO journal 62 11101522
2017 Let-7c-5p inhibits cell proliferation and induces cell apoptosis by targeting ERCC6 in breast cancer. Oncology reports 61 28731186
2007 A novel single nucleotide polymorphism in ERCC6 gene is associated with oral cancer susceptibility in Taiwanese patients. Oral oncology 61 17933579
2001 Requirement for yeast RAD26, a homolog of the human CSB gene, in elongation by RNA polymerase II. Molecular and cellular biology 61 11713297
1993 Structure and expression of the excision repair gene ERCC6, involved in the human disorder Cockayne's syndrome group B. Nucleic acids research 59 8382798
2016 Ultraviolet-B induces ERCC6 repression in lens epithelium cells of age-related nuclear cataract through coordinated DNA hypermethylation and histone deacetylation. Clinical epigenetics 58 27231489
2009 The C-terminal repeat domain of Spt5 plays an important role in suppression of Rad26-independent transcription coupled repair. The Journal of biological chemistry 48 20042611
2002 Yeast RAD26, a homolog of the human CSB gene, functions independently of nucleotide excision repair and base excision repair in promoting transcription through damaged bases. Molecular and cellular biology 44 12024048
2015 Transcription coupled nucleotide excision repair in the yeast Saccharomyces cerevisiae: The ambiguous role of Rad26. DNA repair 36 26429063
2020 Genome-wide role of Rad26 in promoting transcription-coupled nucleotide excision repair in yeast chromatin. Proceedings of the National Academy of Sciences of the United States of America 35 32690696
2002 Fission yeast Rad26 is a regulatory subunit of the Rad3 checkpoint kinase. Molecular biology of the cell 33 11854406
1998 Yeast RNA polymerase II transcription in vitro is inhibited in the presence of nucleotide excision repair: complementation of inhibition by Holo-TFIIH and requirement for RAD26. Molecular and cellular biology 32 9566886
1996 The human CSB (ERCC6) gene corrects the transcription-coupled repair defect in the CHO cell mutant UV61. Nucleic acids research 32 8811084
1992 Localization of the nucleotide excision repair gene ERCC6 to human chromosome 10q11-q21. Genomics 32 1349298
2008 A variant of the Cockayne syndrome B gene ERCC6 confers risk of lung cancer. Human mutation 31 17854076
2007 Cdc18 enforces long-term maintenance of the S phase checkpoint by anchoring the Rad3-Rad26 complex to chromatin. Molecular cell 31 17531813
2004 Cds1 phosphorylation by Rad3-Rad26 kinase is mediated by forkhead-associated domain interaction with Mrc1. The Journal of biological chemistry 30 15173168
2014 Gender and cell-type-specific effects of the transcription-coupled repair protein, ERCC6/CSB, on repeat expansion in a mouse model of the fragile X-related disorders. Human mutation 29 24352881
2009 Significant association of ERCC6 single nucleotide polymorphisms with bladder cancer susceptibility in Taiwan. Anticancer research 28 20044625
2008 ERCC6/CSB gene polymorphisms and lung cancer risk. Cancer letters 28 18789574
1997 A novel mutant allele of Schizosaccharomyces pombe rad26 defective in monitoring S-phase progression to prevent premature mitosis. Molecular and cellular biology 28 9154809
2007 The roles of Rad16 and Rad26 in repairing repressed and actively transcribed genes in yeast. DNA repair 25 17611170
2016 Sen1, the yeast homolog of human senataxin, plays a more direct role than Rad26 in transcription coupled DNA repair. Nucleic acids research 24 27179024
2010 The ERCC6 gene and age-related macular degeneration. PloS one 23 21072178
2000 Excision repair at the level of the nucleotide in the upstream control region, the coding sequence and in the region where transcription terminates of the Saccharomyces cerevisiae MFA2 gene and the role of RAD26. Nucleic acids research 23 10666451
2008 Cockayne syndrome type II in a Druze isolate in Northern Israel in association with an insertion mutation in ERCC6. American journal of medical genetics. Part A 22 18446857
2017 Elevated Expression of ERCC6 Confers Resistance to 5-Fluorouracil and Is Associated with Poor Patient Survival in Colorectal Cancer. DNA and cell biology 21 28665687
2014 The interaction effects of pri-let-7a-1 rs10739971 with PGC and ERCC6 gene polymorphisms in gastric cancer and atrophic gastritis. PloS one 21 24586594
2014 Polymorphism of DNA repair genes OGG1, XRCC1, XPD and ERCC6 in bladder cancer in Belarus. Biomarkers : biochemical indicators of exposure, response, and susceptibility to chemicals 20 25089939
2024 Elf1 promotes Rad26's interaction with lesion-arrested Pol II for transcription-coupled repair. Proceedings of the National Academy of Sciences of the United States of America 18 38194460
2010 ERCC6 founder mutation identified in Finnish patients with COFS syndrome. Clinical genetics 18 20456449
2009 A role for checkpoint kinase-dependent Rad26 phosphorylation in transcription-coupled DNA repair in Saccharomyces cerevisiae. Molecular and cellular biology 18 19901073
2021 MicroRNA Let-7c-5p-Mediated Regulation of ERCC6 Disrupts Autophagic Flux in Age-Related Cataract via the Binding to VCP. Current eye research 17 33703976
2013 The DNA repair gene ERCC6 rs1917799 polymorphism is associated with gastric cancer risk in Chinese. Asian Pacific journal of cancer prevention : APJCP 16 24289633
1999 Rad26, the yeast homolog of the cockayne syndrome B gene product, counteracts inhibition of DNA repair due to RNA polymerase II transcription. The Journal of biological chemistry 16 9880486
2011 Cockayne syndrome caused by paternally inherited 5 Mb deletion of 10q11.2 and a frameshift mutation of ERCC6. European journal of medical genetics 15 21376145
2001 RAD9, RAD24, RAD16 and RAD26 are required for the inducible nucleotide excision repair of UV-induced cyclobutane pyrimidine dimers from the transcribed and non-transcribed regions of the Saccharomyces cerevisiae MFA2 gene. Mutation research 15 11267834
2021 Mechanism of Rad26-assisted rescue of stalled RNA polymerase II in transcription-coupled repair. Nature communications 14 34853308
2015 The transcription-coupled repair protein ERCC6/CSB also protects against repeat expansion in a mouse model of the fragile X premutation. Human mutation 14 25726753
2001 Deletion of the CSB homolog, RAD26, yields Spt(-) strains with proficient transcription-coupled repair. Nucleic acids research 14 11452033
1996 Comparative analyses of relative ERCC3 and ERCC6 mRNA levels in gliomas and adjacent non-neoplastic brain. Molecular carcinogenesis 14 8876669
2025 A Dual Approach with Organoid and CRISPR Screening Reveals ERCC6 as a Determinant of Cisplatin Resistance in Osteosarcoma. Advanced science (Weinheim, Baden-Wurttemberg, Germany) 13 40476445
2018 Novel frame shift mutation in ERCC6 leads to a severe form of Cockayne syndrome with postnatal growth failure and early death: A case report and brief literature review. Medicine 12 30113454
2014 ERCC6 dysfunction presenting as progressive neurological decline with brain hypomyelination. American journal of medical genetics. Part A 12 25251875
2010 A kinase-independent role for the Rad3(ATR)-Rad26(ATRIP) complex in recruitment of Tel1(ATM) to telomeres in fission yeast. PLoS genetics 12 20140190
2004 In UV-irradiated Saccharomyces cerevisiae, overexpression of Swi2/Snf2 family member Rad26 increases transcription-coupled repair and repair of the non-transcribed strand. Molecular microbiology 12 15186415
2011 Maternal origin of a de novo microdeletion spanning the ERCC6 gene in a classic form of the Cockayne syndrome. European journal of medical genetics 11 21477668
2006 Modulation of Rad26- and Rpb9-mediated DNA repair by different promoter elements. The Journal of biological chemistry 11 17023424
2003 Fission yeast Rad26 responds to DNA damage independently of Rad3. BMC genetics 11 12697061
2022 Blockage of ERCC6 Alleviates Spinal Cord Injury Through Weakening Apoptosis, Inflammation, Senescence, and Oxidative Stress. Frontiers in molecular biosciences 10 35392536
2019 Evidence that Moderate Eviction of Spt5 and Promotion of Error-Free Transcriptional Bypass by Rad26 Facilitates Transcription Coupled Nucleotide Excision Repair. Journal of molecular biology 10 30790631
2019 Haplotypes of HTRA1 rs1120638, TIMP3 rs9621532, VEGFA rs833068, CFI rs10033900, ERCC6 rs3793784, and KCTD10 rs56209061 Gene Polymorphisms in Age-Related Macular Degeneration. Disease markers 10 31583032
2019 Histone H4 H75E mutation attenuates global genomic and Rad26-independent transcription-coupled nucleotide excision repair. Nucleic acids research 9 31114907
1994 A possible yeast homolog of human active-gene-repairing helicase ERCC6+. Biochemical and biophysical research communications 9 8198589
2023 Rad2, Rad14 and Rad26 recover Metarhizium robertsii from solar UV damage through photoreactivation in vivo. Microbiological research 8 38154444
2021 Identification and Characterization of a Novel Recurrent ERCC6 Variant in Patients with a Severe Form of Cockayne Syndrome B. Genes 8 34946871
2020 Integrative genomic analysis implicates ERCC6 and its interaction with ERCC8 in susceptibility to breast cancer. Scientific reports 8 33277540
2021 Profiling and Integrated Analysis of the ERCC6-regulated circRNA-miRNA-mRNA Network in Lens Epithelial Cells. Current eye research 7 33632032
2014 Identification of two missense mutations of ERCC6 in three Chinese sisters with Cockayne syndrome by whole exome sequencing. PloS one 7 25463447
2023 Safety and immunogenicity of rAd26 and rAd5 vector-based heterologous prime-boost COVID-19 vaccine against SARS-CoV-2 in healthy adolescents: an open-label, non-randomized, multicenter, phase 1/2, dose-escalation study. Frontiers in immunology 6 37600800
2016 Ocular findings in a patient with Cockayne syndrome with two mutations in the ERCC6 gene. Ophthalmic genetics 6 27186691
2014 A novel ERCC6 splicing variant associated with a mild Cockayne syndrome phenotype. Hormone research in paediatrics 6 25376329
2013 Rad26, the transcription-coupled repair factor in yeast, is required for removal of stalled RNA polymerase-II following UV irradiation. PloS one 6 23991048
2001 The defect in transcription-coupled repair displayed by a Saccharomyces cerevisiae rad26 mutant is dependent on carbon source and is not associated with a lack of transcription. Genetics 6 11454749
2024 The chromatin remodeler ERCC6 and the histone chaperone NAP1 are involved in apurinic/apyrimidinic endonuclease-mediated DNA repair. The Plant cell 5 38367203
2022 A novel heterozygous ERCC6 variant identified in a Chinese family with non-syndromic primary ovarian insufficiency. Molecular genetics & genomic medicine 5 35975393
2021 Strand-specific effect of Rad26 and TFIIS in rescuing transcriptional arrest by CAG trinucleotide repeat slip-outs. Nucleic acids research 5 34197619
2018 The Involvement of ERCC2/XPD and ERCC6/CSB Wild Type Alleles in Protection Against Aging and Cancer. Current aging science 5 28707579
2017 Two novel mutations in ERCC6 cause Cockayne syndrome B in a Chinese family. Molecular medicine reports 5 28440418
2007 Tfb5 is partially dispensable for Rad26 mediated transcription coupled nucleotide excision repair in yeast. DNA repair 5 17644494
2022 Ectopic circSTK39 Expression Ameliorates Hydrogen Peroxide-Induced Human Lens Epithelial Cell Apoptosis and Oxidative Stress through the miR-125a-5p/ERCC6 Pathway. Current eye research 4 36322706
2021 Whole Exome Sequencing Identifies a Novel Homozygous Missense Mutation in the CSB Protein-Encoding ERCC6 Gene in a Taiwanese Boy with Cockayne Syndrome. Life (Basel, Switzerland) 4 34833108
2017 Insights into Rad3 kinase recruitment from the crystal structure of the DNA damage checkpoint protein Rad26. The Journal of biological chemistry 4 28314775
2016 Novel missense mutations in a conserved loop between ERCC6 (CSB) helicase motifs V and VI: Insights into Cockayne syndrome. American journal of medical genetics. Part A 4 26749132
2012 Identification of Two Novel ERCC6 Mutations in Old Order Amish with Cockayne Syndrome. Molecular syndromology 4 23599700
2023 CircMAP3K4 Suppresses H2O2-Induced Human Lens Epithelial Cell Injury by miR-630/ERCC6 Axis in Age-Related Cataract. Current eye research 3 38152055
2022 Whole-exome sequencing revealed a novel ERCC6 variant in a Vietnamese patient with Cockayne syndrome. Human genome variation 3 35668072
2021 Genetic pleiotropy of ERCC6 loss-of-function and deleterious missense variants links retinal dystrophy, arrhythmia, and immunodeficiency in diverse ancestries. Human mutation 3 34005834
2021 Generation of an induced pluripotent stem cell line (IUFi001) from a Cockayne syndrome patient carrying a mutation in the ERCC6 gene. Stem cell research 3 34271225
1999 Neuronal ERCC6 mRNA expression in rat brain induced by a transient focal cerebral ischemia. Zhongguo yao li xue bao = Acta pharmacologica Sinica 3 10437118
2023 ERCC6 plays a promoting role in the progression of non-small cell lung cancer. Biochemistry and cell biology = Biochimie et biologie cellulaire 2 36802454
2022 Genomic analysis of Rad26 and Rad1-Rad10 reveals differences in their dependence on Mediator and RNA polymerase II. Genome research 2 35738899
2024 Preimplantation genetic testing for Cockayne syndrome with a novel ERCC6 variant in a Chinese family. Frontiers in genetics 1 39473441
2017 Meta-analysis reveals no significant association between ERCC6 polymorphisms and bladder cancer risk. The International journal of biological markers 1 27791261

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