Property Summary

NCBI Gene PubMed Count 165
PubMed Score 269.29
PubTator Score 188.26

Knowledge Summary

Patent

No data available

Expression

  Differential Expression (3)

Disease log2 FC p
malignant mesothelioma 1.200 3.8e-06
osteosarcoma -1.191 4.0e-05
tuberculosis -1.300 3.3e-06

Gene RIF (134)

PMID Text
26826127 new role of VCP/p97 segregase in the timely processing of ubiquitinated CSB from damaged chromatin.
26649138 The frequency of the XPD 312Asn allele was significantly higher in T >/= 2 high grade than in T >/= 2 low grade tumors (p = 0.036); the ERCC6 1097Val/Val genotype was strongly associated with muscle-invasive tumors.
26620705 Data suggest that both the most C-terminal region and SUMOylation of a lysine residue in N-terminal region are important for functions of CSB/ERCC6 in transcription-coupled nucleotide excision repair following DNA breakage from UV light.
26616585 Transcription inhibition reduced accumulation of CSB at sites of monoadducts and interstrand crosslinks, but it did not affect recruitment to (although slightly affected retention at) oxidative DNA damage.
26578602 CSB and CTCF can regulate each other's chromatin association, thereby modulating chromatin structure and coordinating gene expression in response to oxidative stress.
26100862 Data indicate that Cockayne syndrome group B protein CSB function is necessary for the recruitment of recombinational factors.
26030138 33 proteins that were not previously known to interact with CSB.
25731027 The polymorphism of DNA repair genes XPD, XRCC1, OGG1, and ERCC6, life expectancy, and the inclination to smoke
25484185 These studies have provided significant functional and mechanistic insights of Rad26p/CSB in regulation of gene expression and genome integrity as described here.
25463447 The present report describes a case of Cockayne syndrome in a Chinese family, with the patients carrying two missense mutations (c.1595A>G, p.Asp532Gly and c.1607T>G, p.Leu536Trp) in the ERCC6 gene in an apparently compound heterozygote.
25376329 new splicing ERCC6 defect causal of Cockayne syndrome.
25251875 ERCC6 dysfunction presenting as progressive neurological decline with brain hypomyelination. This report expands the disease spectrum associated with ERCC6 mutations.
25249633 Data indicate that Cockayne syndrome group B protein CSB is required for transdifferentiation of fibroblasts to neurons.
25136123 CSB-mutated cells, but not UVSSA-deficient cells, have increased levels of intramitochondrial reactive oxygen species (ROS), especially when mitochondrial complex I is inhibited by rotenone.
24928003 Double heterozygote for mutations of ERCC6 is associated with Cockayne syndrome.
24874740 CSB has a crucial role in coordinated regulation of transcription and chromatin remodeling activities that are required during neurogenesis.
24743307 Decreased CSB occupancy of TPA-response elements when c-Jun levels were diminished.
24615090 Results found that polymorphisms in XPG rs2296147 and CSB rs2228526 were significantly associated with prostate cancer susceptibility in the Chinese population analyzed.
24586594 An interaction effect of pri-let-7a-1 rs10739971 polymorphism with ERCC6 rs1917799 polymorphism was observed for the risk of gastric cancer.
24406253 CSB protein stimulates NEIL2 DNA glycosylase activity
24289633 ERCC6 rs1917799 polymorphism is associated with gastric cancer risk.
23733932 In CSB-deficient cells ATF3 remains bound to the promoter, thereby preventing the arrival of polymerase II and the restart of transcription.
23667505 CSB and PCAF play cooperative roles to establish the active state of rRNA genes by histone acetylation
23583689 The review focuses on the participation of the CSB and CSA proteins in many different protein interactions and complexes, and how these interactions inform us about pathways that are defective in the disease.
23562425 CSB has been shown to regulate processes such as the transcriptional recovery after DNA damage, the p53 transcriptional response, the response to hypoxia, the response IGF-1, transactivation of nuclear receptors, transcription of housekeeping genes
23562424 The role of CSA and CSB protein in the oxidative stress response.
23562423 Mitochondrial CSA and CSB: protein interactions and protection from ageing associated DNA mutations.
23422418 Structure, function and regulation of CSB
23419237 In tumor cell lines, CSB is overexpressed and controls cell proliferation and apoptosis.
23322570 Eighteen polymorphisms in four DNA repair genes were genotyped in 789 age related cataract patients and 531 normal controls from the Jiangsu Eye Study.
23253478 Involvement of CSB and XPC in the repair of oxidative DNA lesions independent of the remainder of the nucleotide excision repair reaction.
23229313 CSB modulates the CPT-induced formation of discrete p53-binding protein 1 (53BP1) nuclear foci at sites of transcription-mediated DNA strand breaks
22743267 Patient-derived CSB-deficient cells exhibited a defect in efficient mitochondrial transcript production and that CSB specifically promoted elongation by the mitochondrial RNA polymerase in vitro.
22483866 CSB-PGBD3 fusion protein is important in both health and disease, and could play a role in Cockayne syndrome.
22473955 report an increase in metabolism in the CSB(m/m) mouse model and CSB-deficient cells
22336945 These hypothesis-generating findings suggest that functional variants in ERCC6 may be associated with an increased risk of non-melanoma skin cancer that may be specific to basal cell carcinoma.
22032989 CSA and CSB are identified as the key elements of a regulatory mechanism that equilibrate beneficial and detrimental effects of p53 activity upon cellular stress.
21918225 Testicular nuclear receptor 4 (TR4) regulates UV light-induced responses via Cockayne syndrome B protein-mediated transcription-coupled DNA repair
21852235 the reciprocal regulation of chromatin access by CSB and p53 could be part of a mechanism by which these two proteins coordinate their activities to regulate DNA repair, cell survival, and aging
21756275 BRCA1 polyubiquitinates CSB and this polyubiquitination and subsequent degradation of Cockayne syndrome B protein occur following UV irradiation.
21643987 We suggest a possible novel association between ERCC6 rs4253079 and survival in this group of patients with low-grade and anaplastic gliomas
21566259 Data suggest that Cockayne syndrome B protein protects CAG repeats from expansion by either active reduction of the tract length during parent-child transmission, or by antagonizing the action of OGG1, which tends to promote expansion in somatic cells.
21438118 the disease causing Cockayne syndrome group B protein mutation impairs resolution of pH2AX foci and causes DNA fragility
21072178 Data suggest thatthe AMD-related reduced transcriptional activity of ERCC6 may be caused by diverse, small and heterogeneous genetic and/or environmental determinants.
21072178 Meta-analysis of gene-disease association. (HuGE Navigator)
20813000 Meta-analysis of gene-disease association. (HuGE Navigator)
20673160 Cockayne syndrome B (CSB) protein protects the fetus from xenobiotic-enhanced DNA oxidation and postnatal functional deficits
20541997 Results indicate an essential role for protein ubiquitylation and Cockayne syndrome B's ubiquitin-binding domain in triggering damage incision during transcription-coupled nucleotide excision repair.
20522537 Observational study of gene-disease association. (HuGE Navigator)
20496165 Observational study of gene-disease association and gene-gene interaction. (HuGE Navigator)
20470425 CSB knockdown increased the sensitivity of colorectal and prostate cancer cell lines to cisplatin-induced apoptosis, independent of p53 and MLH1
20456449 ERCC6 founder mutation identified in Finnish patients with COFS syndrome
20453000 Observational study of gene-disease association. (HuGE Navigator)
20379614 Clinical trial of gene-disease association and gene-environment interaction. (HuGE Navigator)
20181933 CSB plays a direct role in mitochondrial base excision repair (BER) by helping recruit, stabilize, and/or retain BER proteins in repair complexes associated with the inner mitochondrial membrane
20122405 The N-terminal region of CSB negatively regulates chromatin association during normal cell growth.
20100872 role in protect from aging- and stress-induced mtDNA mutations and apoptosis-mediated loss of subcutaneous fat, a hallmark of aging
20044625 ERCC6 single nucleotide polymorphism is associated with bladder cancer susceptibility.
20044625 Observational study of gene-disease association. (HuGE Navigator)
19948975 Observational study of gene-disease association, gene-gene interaction, gene-environment interaction, and genetic testing. (HuGE Navigator)
19894250 The 45 published mutations in CSA and CSB to date and 43 new mutations in these genes together with the corresponding clinical data, are reported.
19878615 Observational study of gene-disease association, gene-gene interaction, and gene-environment interaction. (HuGE Navigator)
19789190 Observational study of gene-disease association, gene-gene interaction, and gene-environment interaction. (HuGE Navigator)
19773279 Observational study of gene-disease association. (HuGE Navigator)
19692168 Observational study of gene-disease association. (HuGE Navigator)
19661089 Observational study of gene-disease association. (HuGE Navigator)
19626602 Observational study of gene-disease association. (HuGE Navigator)
19625176 Observational study of gene-disease association. (HuGE Navigator)
19580815 Results describe the ATP-dependent and the ATP-independent biochemical functions of human Cockayne syndrome group B protein (CSB), and show that calcium is a novel metal cofactor of CSB for ATP hydrolysis.
19573080 Observational study of gene-disease association. (HuGE Navigator)
19481676 These results suggest that HNE-DNA adducts are extremely toxic endogenous DNA lesion, and that their processing involves CSB
19444904 Genetic polymorphisms in ERCC6 is associated with Laryngeal cancer risk associated with smoking and alcohol consumption.
19270000 Observational study of gene-disease association. (HuGE Navigator)
19237606 Observational study of gene-disease association, gene-gene interaction, and gene-environment interaction. (HuGE Navigator)
19221478 The CSB protein functions as a master switch factor that can selectively influence the transcription of specific sets of genes, after DNA damage or hypoxia, by influencing the biological functions of Tumor Suppressor Protein p53.
19179336 CSB plays a role in repair of formamidopyrimidines, possibly by interacting with and stimulating NEIL1
19170196 Observational study of gene-disease association and gene-environment interaction. (HuGE Navigator)
19132999 Somatic mutation of ERCC6 is rare in common human cancers in the Korean population.
19132999 Observational study of gene-disease association. (HuGE Navigator)
19116388 Observational study and meta-analysis of gene-disease association. (HuGE Navigator)
19074885 Observational study of gene-disease association. (HuGE Navigator)
19064572 Observational study and meta-analysis of gene-disease association. (HuGE Navigator)
19015224 Observational study of gene-disease association, gene-environment interaction, and pharmacogenomic / toxicogenomic. (HuGE Navigator)
18854777 Observational study of gene-disease association, gene-gene interaction, gene-environment interaction, and pharmacogenomic / toxicogenomic. (HuGE Navigator)
18789574 Genetic polymorphisms of the ERCC6 gene and its role in smoking related lung cancer was studied.
18789574 Observational study of gene-disease association. (HuGE Navigator)
18784753 Data show that CSB mutant cells are unable to react to hypoxic stimuli by properly activating the hypoxia-inducible factor-1 (HIF-1) pathway, a defect that is further enhanced in the event of a concomitant genotoxic stress.
18767034 Observational study of gene-disease association and gene-environment interaction. (HuGE Navigator)
18708399 Observational study of gene-disease association. (HuGE Navigator)
18676680 Observational study of gene-disease association. (HuGE Navigator)
18656484 Data show that truncated Cockayne syndrome B protein represses elongation by RNA polymerase I.
18628313 Description of three additional cases of Cerebro-oculo-facio-skeletal syndrome with novel mutations in the CSB gene.
18446857 a novel insertion mutation, c.1034-1035insT in exon 5 of the ERCC6 gene was identified
18369450 a domesticated PiggyBac-like transposon PGBD3, residing within intron 5 of the CSB gene, functions as an alternative 3' terminal exon
18270339 Observational study and meta-analysis of gene-disease association. (HuGE Navigator)
18185538 a null mutation in the CSB gene may have a role in adult-onset neurological degeneration in a patient with Cockayne syndrome
18183039 Genomic DNA sequencing of the CSB gene showed a homozygous deletion involving non-coding exon 1 and upstream regulatory sequences, but none of the coding exons
18026184 Observational study of gene-disease association and gene-environment interaction. (HuGE Navigator)
17996703 CSB is not required for the ubiquitylation of human RNA polymerase II.
17933579 results firstly suggest that the heterozygous and homozygous A allele of the ERCC6 codon 399 may be associated with the development of oral cancer and may be a novel useful marker for primary prevention and anticancer intervention.
17933579 Observational study of gene-disease association and gene-environment interaction. (HuGE Navigator)
17855454 Observational study of gene-disease association. (HuGE Navigator)
17855454 SNPs associated with prognosis of lung cancer was mapped to ERCC6.
17854076 Observational study of gene-disease association and gene-environment interaction. (HuGE Navigator)
17854076 ERCC6 rs3793784:C>G alters its transcriptional activity and may confer personalized susceptibility to lung cancer.
17728339 Observational study of gene-disease association, gene-gene interaction, and gene-environment interaction. (HuGE Navigator)
17707230 The results reveal the mechanism underlying CSB-mediated activation of ribosomal DNA transcription and link G9a-dependent histone methylation to Pol I transcription elongation through chromatin.
17575242 Observational study of gene-disease association and gene-environment interaction. (HuGE Navigator)
17567611 Purified recombinant Cockayne syndrome B protein and the major human apurinic/apyrimidinic endonuclease, APE1, physically and functionally interact.
17438655 Observational study of gene-disease association and gene-environment interaction. (HuGE Navigator)
17313739 Observational study of gene-disease association. (HuGE Navigator)
17119055 Observational study of gene-disease association and gene-gene interaction. (HuGE Navigator)
16916636 Cockayne Syndrome A and B proteins (CSA) and (CSB) display differential roles in recruitment of transcription-coupled repair (TCR)-specific factors and assembly for TCR occurs without disruption of the UV-stalled RNA polymerase II (RNAPIIo).
16772382 indicate a general role for Cockayne syndrome group B protein (CSB) protein in maintenance and remodeling of chromatin structure and suggest that CS is a disease of transcriptional deregulation caused by misexpression of growth-suppressive
16754848 Observational study of gene-disease association. (HuGE Navigator)
16754848 Results describe age-related macular degeneration (AMD) genetic risk factors through identification of polymorphisms in ERCC6 and in complement factor H.
16751180 examines functional relationship between CSA and CSB in Cockayne syndrome
16601682 Data highlight the pivotal role of CSB in initiating the transcriptional program of certain genes after UV irradiation, and also may explain some of the complex traits of Cockayne syndrome patients.
16537713 Observational study of gene-disease association and gene-gene interaction. (HuGE Navigator)
16492920 Observational study of gene-disease association and gene-environment interaction. (HuGE Navigator)
16399771 Observational study of gene-disease association and gene-environment interaction. (HuGE Navigator)
16258177 Observational study of gene-disease association. (HuGE Navigator)
16246722 The Mutation of Cockayne Syndrome Group B can cause defective transcription-coupled repair and Cockayne syndrome .
16128801 enzymatically active CSB has an apparent molecular mass of approximately 360 kDa, consistent with dimerization of CSB
16107709 results implicate CSB in the PARP-1 poly(ADP-ribosyl)ation response after oxidative stress and thus suggest a novel role of CSB in the cellular response to oxidative damage
15746040 Observational study of gene-disease association, gene-environment interaction, and pharmacogenomic / toxicogenomic. (HuGE Navigator)
15548521 CSB binds DNA as a dimer: DNA wrapping and unwrapping allows CSB to actively alter the DNA double helix conformation, which could influence nucleosomes and other protein-DNA interactions
15486090 no mutation in the CSB cDNA and a normal amount of CSB protein was detected in Kps3, a UV sensitive (UVsS) cell line obtained from an unrelated patient, indicating genetic heterogeneity in UVsS
15226310 DNA damage stabilizes interaction of CSB with the transcription elongation machinery
12606941 CS-B mutant cell lines had deficient transcription after oxidative stress. The CSB protein influenced the transcriptional regulation of certain genes. The ATPase function of CSB is biologically important as the ATPase mutants resemble the CS-B-null ones.
12419226 CSB is a component of RNA pol I transcription
12095617 Cockayne syndrome B protein(ERCC6) enhanced germ cell proliferation arrest and apoptosis, and increased embryonic lethality, suggesting its role in nucleotide excision repair.
12060667 CSB is involved in cellular repair of 8-hydroxyadenine in DNA.
11809892 differential requirement for the ATPase domain of the Cockayne syndrome group B gene in the processing of UV-induced DNA damage and 8-oxoguanine lesions in human cells

AA Sequence

MPNEGIPHSSQTQEQDCLQSQPVSNNEEMAIKQESGGDGEVEEYLSFRSVGDGLSTSAVGCASAAPRRGP      1 - 70
ALLHIDRHQIQAVEPSAQALELQGLGVDVYDQDVLEQGVLQQVDNAIHEASRASQLVDVEKEYRSVLDDL     71 - 140
TSCTTSLRQINKIIEQLSPQAATSRDINRKLDSVKRQKYNKEQQLKKITAKQKHLQAILGGAEVKIELDH    141 - 210
ASLEEDAEPGPSSLGSMLMPVQETAWEELIRTGQMTPFGTQIPQKQEKKPRKIMLNEASGFEKYLADQAK    211 - 280
LSFERKKQGCNKRAARKAPAPVTPPAPVQNKNKPNKKARVLSKKEERLKKHIKKLQKRALQFQGKVGLPK    281 - 350
ARRPWESDMRPEAEGDSEGEESEYFPTEEEEEEEDDEVEGAEADLSGDGTDYELKPLPKGGKRQKKVPVQ    351 - 420
EIDDDFFPSSGEEAEAASVGEGGGGGRKVGRYRDDGDEDYYKQRLRRWNKLRLQDKEKRLKLEDDSEESD    421 - 490
AEFDEGFKVPGFLFKKLFKYQQTGVRWLWELHCQQAGGILGDEMGLGKTIQIIAFLAGLSYSKIRTRGSN    491 - 560
YRFEGLGPTVIVCPTTVMHQWVKEFHTWWPPFRVAILHETGSYTHKKEKLIRDVAHCHGILITSYSYIRL    561 - 630
MQDDISRYDWHYVILDEGHKIRNPNAAVTLACKQFRTPHRIILSGSPMQNNLRELWSLFDFIFPGKLGTL    631 - 700
PVFMEQFSVPITMGGYSNASPVQVKTAYKCACVLRDTINPYLLRRMKSDVKMSLSLPDKNEQVLFCRLTD    701 - 770
EQHKVYQNFVDSKEVYRILNGEMQIFSGLIALRKICNHPDLFSGGPKNLKGLPDDELEEDQFGYWKRSGK    771 - 840
MIVVESLLKIWHKQGQRVLLFSQSRQMLDILEVFLRAQKYTYLKMDGTTTIASRQPLITRYNEDTSIFVF    841 - 910
LLTTRVGGLGVNLTGANRVVIYDPDWNPSTDTQARERAWRIGQKKQVTVYRLLTAGTIEEKIYHRQIFKQ    911 - 980
FLTNRVLKDPKQRRFFKSNDLYELFTLTSPDASQSTETSAIFAGTGSDVQTPKCHLKRRIQPAFGADHDV    981 - 1050
PKRKKFPASNISVNDATSSEEKSEAKGAEVNAVTSNRSDPLKDDPHMSSNVTSNDRLGEETNAVSGPEEL   1051 - 1120
SVISGNGECSNSSGTGKTSMPSGDESIDEKLGLSYKRERPSQAQTEAFWENKQMENNFYKHKSKTKHHSV   1121 - 1190
AEEETLEKHLRPKQKPKNSKHCRDAKFEGTRIPHLVKKRRYQKQDSENKSEAKEQSNDDYVLEKLFKKSV   1191 - 1260
GVHSVMKHDAIMDGASPDYVLVEAEANRVAQDALKALRLSRQRCLGAVSGVPTWTGHRGISGAPAGKKSR   1261 - 1330
FGKKRNSNFSVQHPSSTSPTEKCQDGIMKKEGKDNVPEHFSGRAEDADSSSGPLASSSLLAKMRARNHLI   1331 - 1400
LPERLESESGHLQEASALLPTTEHDDLLVEMRNFIAFQAHTDGQASTREILQEFESKLSASQSCVFRELL   1401 - 1470
RNLCTFHRTSGGEGIWKLKPEYC                                                  1471 - 1493
//

Text Mined References (175)

PMID Year Title
26826127 2016 Valosin-containing Protein (VCP)/p97 Segregase Mediates Proteolytic Processing of Cockayne Syndrome Group B (CSB) in Damaged Chromatin.
26649138 2016 The Cellular Response to Oxidatively Induced DNA Damage and Polymorphism of Some DNA Repair Genes Associated with Clinicopathological Features of Bladder Cancer.
26620705 2016 The C-terminal Region and SUMOylation of Cockayne Syndrome Group B Protein Play Critical Roles in Transcription-coupled Nucleotide Excision Repair.
26616585 2016 Elements That Regulate the DNA Damage Response of Proteins Defective in Cockayne Syndrome.
26578602 2016 The CSB chromatin remodeler and CTCF architectural protein cooperate in response to oxidative stress.
26218421 2015 CSB-PGBD3 Mutations Cause Premature Ovarian Failure.
26100862 2015 DNA damage during the G0/G1 phase triggers RNA-templated, Cockayne syndrome B-dependent homologous recombination.
26030138 2015 Identification of Novel Proteins Co-Purifying with Cockayne Syndrome Group B (CSB) Reveals Potential Roles for CSB in RNA Metabolism and Chromatin Dynamics.
25731027 2014 [The polymorphism of DNA repair genes XPD, XRCC1, OGG1, and ERCC6, life expectancy, and the inclination to smoke].
25484185 2014 Regulation of active genome integrity and expression by Rad26p.
25463447 2014 Identification of two missense mutations of ERCC6 in three Chinese sisters with Cockayne syndrome by whole exome sequencing.
25376329 2014 A novel ERCC6 splicing variant associated with a mild Cockayne syndrome phenotype.
25251875 2014 ERCC6 dysfunction presenting as progressive neurological decline with brain hypomyelination.
25249633 2014 Dysregulation of gene expression as a cause of Cockayne syndrome neurological disease.
25136123 2014 Mitochondrial reactive oxygen species are scavenged by Cockayne syndrome B protein in human fibroblasts without nuclear DNA damage.
24928003 2014 [Genetic analysis for a family with Cockayne syndrome].
24874740 2014 The cockayne syndrome B protein is essential for neuronal differentiation and neuritogenesis.
24743307 2014 The sequence-specific transcription factor c-Jun targets Cockayne syndrome protein B to regulate transcription and chromatin structure.
24615090 2014 Polymorphisms of DNA repair-related genes with susceptibility and prognosis of prostate cancer.
24586594 2014 The interaction effects of pri-let-7a-1 rs10739971 with PGC and ERCC6 gene polymorphisms in gastric cancer and atrophic gastritis.
24406253 2014 Cockayne Syndrome group B protein stimulates NEIL2 DNA glycosylase activity.
24289633 2013 The DNA repair gene ERCC6 rs1917799 polymorphism is associated with gastric cancer risk in Chinese.
23733932 2013 Regulatory interplay of Cockayne syndrome B ATPase and stress-response gene ATF3 following genotoxic stress.
23667505 2013 The chromatin remodeling factor CSB recruits histone acetyltransferase PCAF to rRNA gene promoters in active state for transcription initiation.
23583689 Multiple interaction partners for Cockayne syndrome proteins: implications for genome and transcriptome maintenance.
23562425 Cockayne syndrome group B (CSB) protein: at the crossroads of transcriptional networks.
23562424 The role of CSA and CSB protein in the oxidative stress response.
23562423 Mitochondrial CSA and CSB: protein interactions and protection from ageing associated DNA mutations.
23422418 Structure, function and regulation of CSB: a multi-talented gymnast.
23419237 2013 The CSB repair factor is overexpressed in cancer cells, increases apoptotic resistance, and promotes tumor growth.
23369858 What role (if any) does the highly conserved CSB-PGBD3 fusion protein play in Cockayne syndrome?
23322570 2013 The associations between single nucleotide polymorphisms of DNA repair genes, DNA damage, and age-related cataract: Jiangsu Eye Study.
23253478 2012 Nucleotide excision repair-initiating proteins bind to oxidative DNA lesions in vivo.
23229313 2012 PARP and CSB modulate the processing of transcription-mediated DNA strand breaks.
23186163 2013 Toward a comprehensive characterization of a human cancer cell phosphoproteome.
23028371 2012 Tethering of the conserved piggyBac transposase fusion protein CSB-PGBD3 to chromosomal AP-1 proteins regulates expression of nearby genes in humans.
22743267 2012 Human Cockayne syndrome B protein reciprocally communicates with mitochondrial proteins and promotes transcriptional elongation.
22483866 2012 The conserved Cockayne syndrome B-piggyBac fusion protein (CSB-PGBD3) affects DNA repair and induces both interferon-like and innate antiviral responses in CSB-null cells.
22473955 2012 Cockayne syndrome group B protein prevents the accumulation of damaged mitochondria by promoting mitochondrial autophagy.
22466612 2012 Mutations in UVSSA cause UV-sensitive syndrome and destabilize ERCC6 in transcription-coupled DNA repair.
22466611 2012 UV-sensitive syndrome protein UVSSA recruits USP7 to regulate transcription-coupled repair.
22466610 2012 Mutations in UVSSA cause UV-sensitive syndrome and impair RNA polymerase IIo processing in transcription-coupled nucleotide-excision repair.
22336945 2012 A community-based study of nucleotide excision repair polymorphisms in relation to the risk of non-melanoma skin cancer.
22032989 2011 CSA and CSB proteins interact with p53 and regulate its Mdm2-dependent ubiquitination.
21918225 2011 Testicular nuclear receptor 4 (TR4) regulates UV light-induced responses via Cockayne syndrome B protein-mediated transcription-coupled DNA repair.
21852235 2011 Reciprocally regulated chromatin association of Cockayne syndrome protein B and p53 protein.
21756275 2011 BRCA1 contributes to transcription-coupled repair of DNA damage through polyubiquitination and degradation of Cockayne syndrome B protein.
21643987 2011 Analysis of DNA repair gene polymorphisms and survival in low-grade and anaplastic gliomas.
21566259 2011 Cockayne syndrome B protein antagonizes OGG1 in modulating CAG repeat length in vivo.
21438118 2011 Defective resolution of pH2AX foci and enhanced DNA breakage in ionizing radiation-treated cockayne syndrome B cells.
21406692 2011 System-wide temporal characterization of the proteome and phosphoproteome of human embryonic stem cell differentiation.
21269460 2011 Initial characterization of the human central proteome.
21143350 2011 Mutant Cockayne syndrome group B protein inhibits repair of DNA topoisomerase I-DNA covalent complex.
21072178 2010 The ERCC6 gene and age-related macular degeneration.
20813000 2010 Polymorphisms in DNA repair genes and risk of non-Hodgkin lymphoma in a pooled analysis of three studies.
20673160 2011 Cockayne syndrome B protects against methamphetamine-enhanced oxidative DNA damage in murine fetal brain and postnatal neurodevelopmental deficits.
20541997 2010 A ubiquitin-binding domain in Cockayne syndrome B required for transcription-coupled nucleotide excision repair.
20522537 2010 Variation within DNA repair pathway genes and risk of multiple sclerosis.
20496165 2011 Comprehensive screen of genetic variation in DNA repair pathway genes and postmenopausal breast cancer risk.
20470425 2010 Decreased transcription-coupled nucleotide excision repair capacity is associated with increased p53- and MLH1-independent apoptosis in response to cisplatin.
20456449 2010 ERCC6 founder mutation identified in Finnish patients with COFS syndrome.
20453000 2010 A Large-scale genetic association study of esophageal adenocarcinoma risk.
20379614 Personalized smoking cessation: interactions between nicotine dose, dependence and quit-success genotype score.
20181933 2010 Cockayne syndrome group B protein promotes mitochondrial DNA stability by supporting the DNA repair association with the mitochondrial membrane.
20122405 2010 UV-induced association of the CSB remodeling protein with chromatin requires ATP-dependent relief of N-terminal autorepression.
20100872 2010 Proteins of nucleotide and base excision repair pathways interact in mitochondria to protect from loss of subcutaneous fat, a hallmark of aging.
20068231 2010 Quantitative phosphoproteomics reveals widespread full phosphorylation site occupancy during mitosis.
20044625 2009 Significant association of ERCC6 single nucleotide polymorphisms with bladder cancer susceptibility in Taiwan.
19948975 2009 Integrative predictive model of coronary artery calcification in atherosclerosis.
19894250 2010 Mutation update for the CSB/ERCC6 and CSA/ERCC8 genes involved in Cockayne syndrome.
19878615 2010 Modulation of nucleotide excision repair in human lymphocytes by genetic and dietary factors.
19789190 2009 A gene-based risk score for lung cancer susceptibility in smokers and ex-smokers.
19773279 2009 Association between genetic variants in VEGF, ERCC3 and occupational benzene haematotoxicity.
19692168 2010 Genetic susceptibility to distinct bladder cancer subphenotypes.
19661089 2009 Genetic variation in immune regulation and DNA repair pathways and stomach cancer in China.
19626602 2010 Elevated lung cancer risk is associated with deficiencies in cell cycle checkpoints: genotype and phenotype analyses from a case-control study.
19625176 2009 PTEN identified as important risk factor of chronic obstructive pulmonary disease.
19580815 2009 Nucleic acid binding activity of human Cockayne syndrome B protein and identification of Ca(2+) as a novel metal cofactor.
19573080 2009 Common genetic variants in candidate genes and risk of familial lymphoid malignancies.
19481676 2009 Cockayne syndrome group B protein is engaged in processing of DNA adducts of lipid peroxidation product trans-4-hydroxy-2-nonenal.
19444904 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.
19270000 2009 Genetic susceptibility to esophageal cancer: the role of the nucleotide excision repair pathway.
19237606 2009 Genetic polymorphisms in 85 DNA repair genes and bladder cancer risk.
19221478 2009 Cockayne syndrome B protein (CSB): linking p53, HIF-1 and p300 to robustness, lifespan, cancer and cell fate decisions.
19179336 2009 Cockayne syndrome group B protein stimulates repair of formamidopyrimidines by NEIL1 DNA glycosylase.
19170196 2009 Polymorphisms in innate immunity genes and lung cancer risk in Xuanwei, China.
19132999 2008 Absence of ERCC6 gene mutation in common cancers of Korean patients.
19116388 2009 A field synopsis on low-penetrance variants in DNA repair genes and cancer susceptibility.
19074885 2008 Genetic variants in apoptosis and immunoregulation-related genes are associated with risk of chronic lymphocytic leukemia.
19064572 2008 Polymorphism in the IL18 gene and epithelial ovarian cancer in non-Hispanic white women.
19015224 2009 Polymorphisms in C2, CFB and C3 are associated with progression to advanced age related macular degeneration associated with visual loss.
18987736 2008 DNA sequencing of a cytogenetically normal acute myeloid leukaemia genome.
18854777 2008 Germline genetic variations in drug action pathways predict clinical outcomes in advanced lung cancer treated with platinum-based chemotherapy.
18789574 2009 ERCC6/CSB gene polymorphisms and lung cancer risk.
18784753 2008 CSB protein is (a direct target of HIF-1 and) a critical mediator of the hypoxic response.
18767034 2008 Nucleotide excision repair polymorphisms may modify ionizing radiation-related breast cancer risk in US radiologic technologists.
18708399 2008 Association between common genetic variation in Cockayne syndrome A and B genes and nucleotide excision repair capacity among smokers.
18676680 2008 Pathway-based evaluation of 380 candidate genes and lung cancer susceptibility suggests the importance of the cell cycle pathway.
18669648 2008 A quantitative atlas of mitotic phosphorylation.
18656484 2008 Truncated Cockayne syndrome B protein represses elongation by RNA polymerase I.
18628313 2008 Cerebro-oculo-facio-skeletal syndrome: three additional cases with CSB mutations, new diagnostic criteria and an approach to investigation.
18446857 2008 Cockayne syndrome type II in a Druze isolate in Northern Israel in association with an insertion mutation in ERCC6.
18438403 2008 Protein lysine methyltransferase G9a acts on non-histone targets.
18369450 2008 An abundant evolutionarily conserved CSB-PiggyBac fusion protein expressed in Cockayne syndrome.
18270339 2008 Comprehensive analysis of DNA repair gene variants and risk of meningioma.
18185538 2008 Adult-onset neurological degeneration in a patient with Cockayne syndrome and a null mutation in the CSB gene.
18183039 2008 Deletion of 5' sequences of the CSB gene provides insight into the pathophysiology of Cockayne syndrome.
18026184 2008 NAT2 and NER genetic variants and sporadic prostate cancer susceptibility in African Americans.
17996703 2007 Damage-induced ubiquitylation of human RNA polymerase II by the ubiquitin ligase Nedd4, but not Cockayne syndrome proteins or BRCA1.
17933579 2008 A novel single nucleotide polymorphism in ERCC6 gene is associated with oral cancer susceptibility in Taiwanese patients.
17855454 2007 Genetic variation in the DNA repair genes is predictive of outcome in lung cancer.
17854076 2008 A variant of the Cockayne syndrome B gene ERCC6 confers risk of lung cancer.
17728339 2007 High-order interactions among genetic polymorphisms in nucleotide excision repair pathway genes and smoking in modulating bladder cancer risk.
17707230 2007 Activation of RNA polymerase I transcription by cockayne syndrome group B protein and histone methyltransferase G9a.
17626041 2007 Cockayne syndrome protein B interacts with and is phosphorylated by c-Abl tyrosine kinase.
17575242 2007 Nucleotide excision repair pathway genes and oral premalignant lesions.
17567611 2007 Cockayne syndrome B protein stimulates apurinic endonuclease 1 activity and protects against agents that introduce base excision repair intermediates.
17438655 Association between nucleotide excision repair gene polymorphisms and chromosomal damage in coke-oven workers.
17313739 2006 [Association between nucleotide excision repair gene polymorphisms and chromosomal damage in coke-oven workers].
17119055 2006 Genetic variation in the nucleotide excision repair pathway and colorectal cancer risk.
16964243 2006 A probability-based approach for high-throughput protein phosphorylation analysis and site localization.
16959974 2006 The consensus coding sequences of human breast and colorectal cancers.
16916636 2006 Cockayne syndrome A and B proteins differentially regulate recruitment of chromatin remodeling and repair factors to stalled RNA polymerase II in vivo.
16772382 2006 Cockayne syndrome group B protein (CSB) plays a general role in chromatin maintenance and remodeling.
16754848 2006 Synergic effect of polymorphisms in ERCC6 5' flanking region and complement factor H on age-related macular degeneration predisposition.
16751180 2006 CSA-dependent degradation of CSB by the ubiquitin-proteasome pathway establishes a link between complementation factors of the Cockayne syndrome.
16603771 2006 The WSTF-SNF2h chromatin remodeling complex interacts with several nuclear proteins in transcription.
16601682 2006 Cockayne syndrome B protein regulates the transcriptional program after UV irradiation.
16565220 2006 Phosphoproteome analysis of the human mitotic spindle.
16537713 2006 Genetic variation in the nucleotide excision repair pathway and bladder cancer risk.
16492920 2006 Nucleotide excision repair gene polymorphisms and risk of advanced colorectal adenoma: XPC polymorphisms modify smoking-related risk.
16399771 2006 Polymorphisms in nucleotide excision repair genes, smoking and breast cancer in African Americans and whites: a population-based case-control study.
16344560 2006 Diversification of transcriptional modulation: large-scale identification and characterization of putative alternative promoters of human genes.
16258177 2006 Polymorphisms in nucleotide excision repair genes and risk of multiple primary melanoma: the Genes Environment and Melanoma Study.
16246722 2005 Recognition of RNA polymerase II and transcription bubbles by XPG, CSB, and TFIIH: insights for transcription-coupled repair and Cockayne Syndrome.
16128801 2005 The Cockayne syndrome group B protein is a functional dimer.
16107709 2005 Cooperation of the Cockayne syndrome group B protein and poly(ADP-ribose) polymerase 1 in the response to oxidative stress.
15746040 2005 Nucleotide excision repair gene polymorphisms and recurrence after treatment for superficial bladder cancer.
15548521 2005 The CSB protein actively wraps DNA.
15486090 2004 Complete absence of Cockayne syndrome group B gene product gives rise to UV-sensitive syndrome but not Cockayne syndrome.
15231748 2004 Functional proteomics mapping of a human signaling pathway.
15226310 2004 DNA damage stabilizes interaction of CSB with the transcription elongation machinery.
15164054 2004 The DNA sequence and comparative analysis of human chromosome 10.
14702039 2004 Complete sequencing and characterization of 21,243 full-length human cDNAs.
12606941 2003 The transcriptional response after oxidative stress is defective in Cockayne syndrome group B cells.
12560492 2003 Functional consequences of mutations in the conserved SF2 motifs and post-translational phosphorylation of the CSB protein.
12477932 2002 Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.
12419226 2002 CSB is a component of RNA pol I transcription.
12121485 2002 Neuroprotection by melatonin against ischemic neuronal injury associated with modulation of DNA damage and repair in the rat following a transient cerebral ischemia.
12060667 2002 The cockayne syndrome group B gene product is involved in cellular repair of 8-hydroxyadenine in DNA.
11313499 2001 Structural basis of transcription: an RNA polymerase II elongation complex at 3.3 A resolution.
10944529 2000 XAB2, a novel tetratricopeptide repeat protein involved in transcription-coupled DNA repair and transcription.
10882116 2000 Activation of p53 or loss of the Cockayne syndrome group B repair protein causes metaphase fragility of human U1, U2, and 5S genes.
10767341 2000 Identical mutations in the CSB gene associated with either Cockayne syndrome or the DeSanctis-cacchione variant of xeroderma pigmentosum.
10739753 2000 Manitoba aboriginal kindred with original cerebro-oculo- facio-skeletal syndrome has a mutation in the Cockayne syndrome group B (CSB) gene.
10564257 1999 The ATPase domain but not the acidic region of Cockayne syndrome group B gene product is essential for DNA repair.
10498399 1999 Expression in normal human tissues of five nucleotide excision repair genes measured simultaneously by multiplex reverse transcription-polymerase chain reaction.
10447254 1999 A summary of mutations in the UV-sensitive disorders: xeroderma pigmentosum, Cockayne syndrome, and trichothiodystrophy.
10196384 1999 Alterations in the CSB gene in three Italian patients with the severe form of Cockayne syndrome (CS) but without clinical photosensitivity.
9973627 1999 Repair of 8-oxoguanine in DNA is deficient in Cockayne syndrome group B cells.
9774388 1998 RNA polymerase II elongation complexes containing the Cockayne syndrome group B protein interact with a molecular complex containing the transcription factor IIH components xeroderma pigmentosum B and p62.
9450932 1998 S-phase-specific activation of Cds1 kinase defines a subpathway of the checkpoint response in Schizosaccharomyces pombe.
9443879 1998 Molecular analysis of mutations in the CSB (ERCC6) gene in patients with Cockayne syndrome.
9372911 1997 Recruitment of the putative transcription-repair coupling factor CSB/ERCC6 to RNA polymerase II elongation complexes.
9326587 1997 Cockayne syndrome group B protein enhances elongation by RNA polymerase II.
9137418 Nuclear import of DNA repair proteins.
8999876 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.
8652557 1996 Interactions involving the human RNA polymerase II transcription/nucleotide excision repair complex TFIIH, the nucleotide excision repair protein XPG, and Cockayne syndrome group B (CSB) protein.
8382798 1993 Structure and expression of the excision repair gene ERCC6, involved in the human disorder Cockayne's syndrome group B.
7664335 1995 The Cockayne syndrome group A gene encodes a WD repeat protein that interacts with CSB protein and a subunit of RNA polymerase II TFIIH.
7663514 1995 p53 modulation of TFIIH-associated nucleotide excision repair activity.
2172786 1990 Molecular cloning of the human DNA excision repair gene ERCC-6.
1951442 1991 Apparent late-onset Cockayne syndrome and interstitial deletion of the long arm of chromosome 10 (del(10)(q11.23q21.2)).
1349298 1992 Localization of the nucleotide excision repair gene ERCC6 to human chromosome 10q11-q21.
1339317 1992 ERCC6, a member of a subfamily of putative helicases, is involved in Cockayne's syndrome and preferential repair of active genes.