XB-ART-41005
DNA Repair (Amst)
2010 Mar 02;93:331-44. doi: 10.1016/j.dnarep.2009.12.011.
Show Gene links
Show Anatomy links
Roles of Werner syndrome protein in protection of genome integrity.
Rossi ML
,
Ghosh AK
,
Bohr VA
.
???displayArticle.abstract???
Werner syndrome protein (WRN) is one of a family of five human RecQ helicases implicated in the maintenance of genome stability. The conserved RecQ family also includes RecQ1, Bloom syndrome protein (BLM), RecQ4, and RecQ5 in humans, as well as Sgs1 in Saccharomyces cerevisiae, Rqh1 in Schizosaccharomyces pombe, and homologs in Caenorhabditis elegans, Xenopus laevis, and Drosophila melanogaster. Defects in three of the RecQ helicases, RecQ4, BLM, and WRN, cause human pathologies linked with cancer predisposition and premature aging. Mutations in the WRN gene are the causative factor of Werner syndrome (WS). WRN is one of the best characterized of the RecQ helicases and is known to have roles in DNA replication and repair, transcription, and telomere maintenance. Studies both in vitro and in vivo indicate that the roles of WRN in a variety of DNA processes are mediated by post-translational modifications, as well as several important protein-protein interactions. In this work, we will summarize some of the early studies on the cellular roles of WRN and highlight the recent findings that shed some light on the link between the protein with its cellular functions and the disease pathology.
???displayArticle.pubmedLink??? 20075015
???displayArticle.pmcLink??? PMC2827637
???displayArticle.link??? DNA Repair (Amst)
???displayArticle.grants??? [+]
Z01 AG000726-16 NIA NIH HHS , Z01 AG000726-16 Intramural NIH HHS
Species referenced: Xenopus laevis
Genes referenced: blm recql recql4 recql5 wrn
References [+] :
Abraham,
Cell cycle checkpoint signaling through the ATM and ATR kinases.
2001, Pubmed
Abraham, Cell cycle checkpoint signaling through the ATM and ATR kinases. 2001, Pubmed
Aggarwal, Premature aging syndrome gene WRN genetically interacts with a topoisomerase. 2009, Pubmed
Agrelo, Epigenetic inactivation of the premature aging Werner syndrome gene in human cancer. 2006, Pubmed
Ahn, Regulation of WRN helicase activity in human base excision repair. 2004, Pubmed
Aygün, A RECQ5-RNA polymerase II association identified by targeted proteomic analysis of human chromatin. 2008, Pubmed
Aygün, Direct inhibition of RNA polymerase II transcription by RECQL5. 2009, Pubmed
Bachrati, RecQ helicases: guardian angels of the DNA replication fork. 2008, Pubmed
Balajee, The Werner syndrome protein is involved in RNA polymerase II transcription. 1999, Pubmed
Bartek, Checking on DNA damage in S phase. 2004, Pubmed
Baynton, WRN interacts physically and functionally with the recombination mediator protein RAD52. 2003, Pubmed
Blackburn, The molecular structure of centromeres and telomeres. 1984, Pubmed
Blander, Physical and functional interaction between p53 and the Werner's syndrome protein. 1999, Pubmed
Blander, DNA damage-induced translocation of the Werner helicase is regulated by acetylation. 2002, Pubmed
Blank, The Werner syndrome protein confers resistance to the DNA lesions N3-methyladenine and O6-methylguanine: implications for WRN function. 2004, Pubmed
Bohr, Rising from the RecQ-age: the role of human RecQ helicases in genome maintenance. 2008, Pubmed
Bohr, Werner syndrome protein 1367 variants and disposition towards coronary artery disease in Caucasian patients. 2004, Pubmed
Bohr, Repair of oxidative DNA damage in nuclear and mitochondrial DNA, and some changes with aging in mammalian cells. 2002, Pubmed
Boubriak, DmWRNexo is a 3'-5' exonuclease: phenotypic and biochemical characterization of mutants of the Drosophila orthologue of human WRN exonuclease. 2009, Pubmed
Bridgewater, Arrest of replication by mammalian DNA polymerases alpha and beta caused by chromium-DNA lesions. 1998, Pubmed
Brosh, Human premature aging, DNA repair and RecQ helicases. 2007, Pubmed
Brosh, Functional and physical interaction between WRN helicase and human replication protein A. 1999, Pubmed
Brosh, Replication protein A physically interacts with the Bloom's syndrome protein and stimulates its helicase activity. 2000, Pubmed
Brosh, p53 Modulates the exonuclease activity of Werner syndrome protein. 2001, Pubmed
Brosh, Werner syndrome protein interacts with human flap endonuclease 1 and stimulates its cleavage activity. 2001, Pubmed
Brosh, Biochemical characterization of the WRN-FEN-1 functional interaction. 2002, Pubmed
Bukowy, WRN Exonuclease activity is blocked by specific oxidatively induced base lesions positioned in either DNA strand. 2008, Pubmed
Casper, ATR regulates fragile site stability. 2002, Pubmed
Castro, WRN polymorphisms affect expression levels of plasminogen activator inhibitor type 1 in cultured fibroblasts. 2008, Pubmed
Castro, Polymorphisms at the Werner locus: II. 1074Leu/Phe, 1367Cys/Arg, longevity, and atherosclerosis. 2000, Pubmed
Celli, Ku70 stimulates fusion of dysfunctional telomeres yet protects chromosome ends from homologous recombination. 2006, Pubmed
Champoux, DNA topoisomerases: structure, function, and mechanism. 2001, Pubmed
Chang, Essential role of limiting telomeres in the pathogenesis of Werner syndrome. 2004, Pubmed
Chen, Evidence for a replication function of FFA-1, the Xenopus orthologue of Werner syndrome protein. 2001, Pubmed , Xenbase
Chen, LMNA mutations in atypical Werner's syndrome. 2003, Pubmed
Cheng, WRN is required for ATM activation and the S-phase checkpoint in response to interstrand cross-link-induced DNA double-strand breaks. 2008, Pubmed
Cheng, Collaboration of Werner syndrome protein and BRCA1 in cellular responses to DNA interstrand cross-links. 2006, Pubmed
Cheng, Werner syndrome protein phosphorylation by abl tyrosine kinase regulates its activity and distribution. 2003, Pubmed
Cheng, Linkage between Werner syndrome protein and the Mre11 complex via Nbs1. 2004, Pubmed
Choudhary, Biochemical and kinetic characterization of the DNA helicase and exonuclease activities of werner syndrome protein. 2004, Pubmed
Christmann, WRN protects against topo I but not topo II inhibitors by preventing DNA break formation. 2008, Pubmed
Chu, RecQ helicases: multifunctional genome caretakers. 2009, Pubmed
Cohen, Recombination-mediated lengthening of terminal telomeric repeats requires the Sgs1 DNA helicase. 2001, Pubmed
Compton, The Werner syndrome protein binds replication fork and holliday junction DNAs as an oligomer. 2008, Pubmed
Constantinou, Werner's syndrome protein (WRN) migrates Holliday junctions and co-localizes with RPA upon replication arrest. 2000, Pubmed
Cooper, Ku complex interacts with and stimulates the Werner protein. 2000, Pubmed
Cox, Modeling Werner Syndrome in Drosophila melanogaster: hyper-recombination in flies lacking WRN-like exonuclease. 2007, Pubmed
Crabbe, Defective telomere lagging strand synthesis in cells lacking WRN helicase activity. 2004, Pubmed
Crabbe, In the end, it's all structure. 2005, Pubmed
Crabbe, Telomere dysfunction as a cause of genomic instability in Werner syndrome. 2007, Pubmed
Cui, Characterization of the DNA-unwinding activity of human RECQ1, a helicase specifically stimulated by human replication protein A. 2003, Pubmed
Cui, Analysis of the unwinding activity of the dimeric RECQ1 helicase in the presence of human replication protein A. 2004, Pubmed
Dang, The c-Myc target gene network. 2006, Pubmed
Das, The human Werner syndrome protein stimulates repair of oxidative DNA base damage by the DNA glycosylase NEIL1. 2007, Pubmed
Dhillon, Functional role of the Werner syndrome RecQ helicase in human fibroblasts. 2007, Pubmed
Dianov, Role of DNA polymerase beta in the excision step of long patch mammalian base excision repair. 1999, Pubmed
Dietschy, The molecular role of the Rothmund-Thomson-, RAPADILINO- and Baller-Gerold-gene product, RECQL4: recent progress. 2007, Pubmed
Ding, Model of human aging: recent findings on Werner's and Hutchinson-Gilford progeria syndromes. 2008, Pubmed
Ding, Genetic variation in the premature aging gene WRN: a case-control study on breast cancer susceptibility. 2007, Pubmed
Doh, Novel LMNA gene mutation in a patient with Atypical Werner's Syndrome. 2009, Pubmed
Doherty, Physical and functional mapping of the replication protein a interaction domain of the werner and bloom syndrome helicases. 2005, Pubmed , Xenbase
Dominguez-Sola, Non-transcriptional control of DNA replication by c-Myc. 2007, Pubmed , Xenbase
Du, Telomere shortening exposes functions for the mouse Werner and Bloom syndrome genes. 2004, Pubmed
Epstein, Werner's syndrome a review of its symptomatology, natural history, pathologic features, genetics and relationship to the natural aging process. 1966, Pubmed
Esteller, Epigenetic gene silencing in cancer: the DNA hypermethylome. 2007, Pubmed
Fortini, 8-Oxoguanine DNA damage: at the crossroad of alternative repair pathways. 2003, Pubmed
Franchitto, Replication fork stalling in WRN-deficient cells is overcome by prompt activation of a MUS81-dependent pathway. 2008, Pubmed
Friedberg, DNA repair: from molecular mechanism to human disease. 2006, Pubmed
Fry, Human werner syndrome DNA helicase unwinds tetrahelical structures of the fragile X syndrome repeat sequence d(CGG)n. 1999, Pubmed
Fujiwara, A retarded rate of DNA replication and normal level of DNA repair in Werner's syndrome fibroblasts in culture. 1977, Pubmed
Fujiwara, Abnormal fibroblast aging and DNA replication in the Werner syndrome. 1985, Pubmed
Futami, Role of Werner syndrome gene product helicase in carcinogenesis and in resistance to genotoxins by cancer cells. 2008, Pubmed
Futami, Increased chemotherapeutic activity of camptothecin in cancer cells by siRNA-induced silencing of WRN helicase. 2007, Pubmed
GELLERT, Helix formation by guanylic acid. 1962, Pubmed
Galván, Depletion of WRN enhances DNA damage in HeLa cells exposed to the benzene metabolite, hydroquinone. 2008, Pubmed
Garcia, Human RECQ5beta, a protein with DNA helicase and strand-annealing activities in a single polypeptide. 2004, Pubmed
Gebhart, Spontaneous and induced chromosomal instability in Werner syndrome. 1988, Pubmed
German, Bloom syndrome: a mendelian prototype of somatic mutational disease. 1993, Pubmed
German, Bloom's syndrome. 1995, Pubmed
Ghosh, Telomeric D-loops containing 8-oxo-2'-deoxyguanosine are preferred substrates for Werner and Bloom syndrome helicases and are bound by POT1. 2009, Pubmed
Glover, Mechanisms of common fragile site instability. 2005, Pubmed
Glover, DNA polymerase alpha inhibition by aphidicolin induces gaps and breaks at common fragile sites in human chromosomes. 1984, Pubmed
Goto, Hierarchical deterioration of body systems in Werner's syndrome: implications for normal ageing. 1997, Pubmed
Gray, The Werner syndrome protein is a DNA helicase. 1997, Pubmed
Gray, Werner helicase is localized to transcriptionally active nucleoli of cycling cells. 1998, Pubmed
Greenberg, Telomeres, crisis and cancer. 2005, Pubmed
Greider, Telomeres, telomerase and cancer. 1996, Pubmed
Griffith, Mammalian telomeres end in a large duplex loop. 1999, Pubmed
Grigorova, Spontaneous and X-ray-induced chromosomal aberrations in Werner syndrome cells detected by FISH using chromosome-specific painting probes. 2000, Pubmed
Ha, Generation of S phase-dependent DNA double-strand breaks by Cr(VI) exposure: involvement of ATM in Cr(VI) induction of gamma-H2AX. 2004, Pubmed
Hanada, Molecular genetics of RecQ helicase disorders. 2007, Pubmed
Hanaoka, Decrease in the average size of replicons in a Werner syndrome cell line by Simian virus 40 infection. 1983, Pubmed
Hanaoka, Autoradiographic studies of DNA replication in Werner's syndrome cells. 1985, Pubmed
Harley, Telomeres shorten during ageing of human fibroblasts. 1990, Pubmed
Harrigan, The Werner syndrome protein operates in base excision repair and cooperates with DNA polymerase beta. 2006, Pubmed
Harrigan, WRN exonuclease activity is blocked by DNA termini harboring 3' obstructive groups. 2007, Pubmed
Harrigan, Metal-catalyzed oxidation of the Werner syndrome protein causes loss of catalytic activities and impaired protein-protein interactions. 2007, Pubmed
Harrigan, The Werner syndrome protein stimulates DNA polymerase beta strand displacement synthesis via its helicase activity. 2003, Pubmed
Hirai, WRN gene 1367 Arg allele protects against development of type 2 diabetes mellitus. 2005, Pubmed
Huang, The spectrum of WRN mutations in Werner syndrome patients. 2006, Pubmed
Huang, The premature ageing syndrome protein, WRN, is a 3'-->5' exonuclease. 1998, Pubmed
Huang, Characterization of the human and mouse WRN 3'-->5' exonuclease. 2000, Pubmed
Hyun, Biochemical characterization of the WRN-1 RecQ helicase of Caenorhabditis elegans. 2008, Pubmed
Imamura, Werner and Bloom helicases are involved in DNA repair in a complementary fashion. 2002, Pubmed
Imoto, Synthesis, DNA polymerase incorporation, and enzymatic phosphate hydrolysis of formamidopyrimidine nucleoside triphosphates. 2006, Pubmed
Izumikawa, Association of human DNA helicase RecQ5beta with RNA polymerase II and its possible role in transcription. 2008, Pubmed
Jacob, Phenotypic heterogeneity in body fat distribution in patients with atypical Werner's syndrome due to heterozygous Arg133Leu lamin A/C mutation. 2005, Pubmed
Kahyo, Sirtuin-mediated deacetylation pathway stabilizes Werner syndrome protein. 2008, Pubmed
Kamath-Loeb, The enzymatic activities of the Werner syndrome protein are disabled by the amino acid polymorphism R834C. 2004, Pubmed
Kamath-Loeb, Functional interaction between the Werner Syndrome protein and DNA polymerase delta. 2000, Pubmed
Kamath-Loeb, Werner syndrome protein. II. Characterization of the integral 3' --> 5' DNA exonuclease. 1998, Pubmed
Kamath-Loeb, Interactions between the Werner syndrome helicase and DNA polymerase delta specifically facilitate copying of tetraplex and hairpin structures of the d(CGG)n trinucleotide repeat sequence. 2001, Pubmed
Karmakar, Werner protein is a target of DNA-dependent protein kinase in vivo and in vitro, and its catalytic activities are regulated by phosphorylation. 2002, Pubmed
Karmakar, Ku heterodimer binds to both ends of the Werner protein and functional interaction occurs at the Werner N-terminus. 2002, Pubmed
Kawanishi, Site-specific oxidation at GG and GGG sequences in double-stranded DNA by benzoyl peroxide as a tumor promoter. 1999, Pubmed
Kellermayer, The versatile RECQL4. 2006, Pubmed
Kitao, Mutations in RECQL4 cause a subset of cases of Rothmund-Thomson syndrome. 1999, Pubmed
Kruk, DNA damage and repair in telomeres: relation to aging. 1995, Pubmed
Kusumoto, Werner protein cooperates with the XRCC4-DNA ligase IV complex in end-processing. 2008, Pubmed
Kusumoto, The role of WRN in DNA repair is affected by post-translational modifications. 2007, Pubmed , Xenbase
Kyng, Gene expression profiling in Werner syndrome closely resembles that of normal aging. 2003, Pubmed
Kääriäinen, RAPADILINO syndrome with radial and patellar aplasia/hypoplasia as main manifestations. 1989, Pubmed
Laine, Werner protein stimulates topoisomerase I DNA relaxation activity. 2003, Pubmed
Lan, Accumulation of Werner protein at DNA double-strand breaks in human cells. 2005, Pubmed
Lebel, The Werner syndrome gene product co-purifies with the DNA replication complex and interacts with PCNA and topoisomerase I. 1999, Pubmed
Lee, A Werner syndrome protein homolog affects C. elegans development, growth rate, life span and sensitivity to DNA damage by acting at a DNA damage checkpoint. 2004, Pubmed
Leppard, Human DNA topoisomerase I: relaxation, roles, and damage control. 2005, Pubmed
Levy, Telomere end-replication problem and cell aging. 1992, Pubmed
Li, Regulation of WRN protein cellular localization and enzymatic activities by SIRT1-mediated deacetylation. 2008, Pubmed
Li, WRN controls formation of extrachromosomal telomeric circles and is required for TRF2DeltaB-mediated telomere shortening. 2008, Pubmed
Li, Requirements for the nucleolytic processing of DNA ends by the Werner syndrome protein-Ku70/80 complex. 2001, Pubmed
Li, Inhibition of the Bloom's and Werner's syndrome helicases by G-quadruplex interacting ligands. 2001, Pubmed
Li, Displacement of DNA-PKcs from DNA ends by the Werner syndrome protein. 2002, Pubmed
Lindahl, Instability and decay of the primary structure of DNA. 1993, Pubmed
Liu, The Werner syndrome protein functions in repair of Cr(VI)-induced replication-associated DNA damage. 2009, Pubmed
Lombard, Mutations in the WRN gene in mice accelerate mortality in a p53-null background. 2000, Pubmed
Lutomska, The transcriptional response to distinct growth factors is impaired in Werner syndrome cells. 2008, Pubmed
Machwe, RecQ family members combine strand pairing and unwinding activities to catalyze strand exchange. 2005, Pubmed
Machwe, Accelerated methylation of ribosomal RNA genes during the cellular senescence of Werner syndrome fibroblasts. 2000, Pubmed
Mandell, Expression of a RecQ helicase homolog affects progression through crisis in fission yeast lacking telomerase. 2005, Pubmed
Michishita, SIRT6 is a histone H3 lysine 9 deacetylase that modulates telomeric chromatin. 2008, Pubmed
Mohaghegh, The Bloom's and Werner's syndrome proteins are DNA structure-specific helicases. 2001, Pubmed
Muftuoglu, Acetylation regulates WRN catalytic activities and affects base excision DNA repair. 2008, Pubmed
Muftuoglu, The clinical characteristics of Werner syndrome: molecular and biochemical diagnosis. 2008, Pubmed
Multani, WRN at telomeres: implications for aging and cancer. 2007, Pubmed
Murano, Increased blood plasminogen activator inhibitor-1 and intercellular adhesion molecule-1 as possible risk factors of atherosclerosis in Werner syndrome. 1997, Pubmed
Nakayama, Association of a missense single nucleotide polymorphism, Cys1367Arg of the WRN gene, with the risk of bone and soft tissue sarcomas in Japan. 2008, Pubmed
Newman, Short dysfunctional telomeres impair the repair of arsenite-induced oxidative damage in mouse cells. 2008, Pubmed
O'Brien, Critical role of chromium (Cr)-DNA interactions in the formation of Cr-induced polymerase arresting lesions. 2002, Pubmed
Ogburn, An apoptosis-inducing genotoxin differentiates heterozygotic carriers for Werner helicase mutations from wild-type and homozygous mutants. 1997, Pubmed
Olovnikov, [Principle of marginotomy in template synthesis of polynucleotides]. 1971, Pubmed
Opresko, POT1 stimulates RecQ helicases WRN and BLM to unwind telomeric DNA substrates. 2005, Pubmed
Opresko, Role for the Werner syndrome protein in the promotion of tumor cell growth. 2007, Pubmed
Opresko, Telomere ResQue and preservation--roles for the Werner syndrome protein and other RecQ helicases. 2008, Pubmed
Opresko, The Werner syndrome helicase and exonuclease cooperate to resolve telomeric D loops in a manner regulated by TRF1 and TRF2. 2004, Pubmed
Opresko, Telomere-binding protein TRF2 binds to and stimulates the Werner and Bloom syndrome helicases. 2002, Pubmed
Opresko, Werner syndrome and the function of the Werner protein; what they can teach us about the molecular aging process. 2003, Pubmed
Orren, A functional interaction of Ku with Werner exonuclease facilitates digestion of damaged DNA. 2001, Pubmed
Orren, The Werner syndrome helicase/exonuclease (WRN) disrupts and degrades D-loops in vitro. 2002, Pubmed
Otterlei, Werner syndrome protein participates in a complex with RAD51, RAD54, RAD54B and ATR in response to ICL-induced replication arrest. 2006, Pubmed
Ozsoy, Analysis of helicase activity and substrate specificity of Drosophila RECQ5. 2003, Pubmed
Paeschke, Telomere end-binding proteins control the formation of G-quadruplex DNA structures in vivo. 2005, Pubmed
Parkinson, Crystal structure of parallel quadruplexes from human telomeric DNA. 2002, Pubmed
Pichierri, Werner's syndrome protein is required for correct recovery after replication arrest and DNA damage induced in S-phase of cell cycle. 2001, Pubmed
Pichierri, Werner's syndrome protein is phosphorylated in an ATR/ATM-dependent manner following replication arrest and DNA damage induced during the S phase of the cell cycle. 2003, Pubmed
Pinto, WRN Cys1367Arg SNP is not associated with risk and prognosis of gliomas in Southeast Brazil. 2008, Pubmed
Pirzio, Werner syndrome helicase activity is essential in maintaining fragile site stability. 2008, Pubmed
Poot, Impaired S-phase transit of Werner syndrome cells expressed in lymphoblastoid cell lines. 1992, Pubmed
Poot, Werner syndrome lymphoblastoid cells are sensitive to camptothecin-induced apoptosis in S-phase. 1999, Pubmed
Poot, Werner syndrome cells are sensitive to DNA cross-linking drugs. 2001, Pubmed
Popuri, The Human RecQ helicases, BLM and RECQ1, display distinct DNA substrate specificities. 2008, Pubmed
Randall, Structure of long telomeric RNA transcripts: the G-rich RNA forms a compact repeating structure containing G-quartets. 2009, Pubmed
Ren, Werner syndrome protein, WRN, protects cells from DNA damage induced by the benzene metabolite hydroquinone. 2009, Pubmed
Renard, Novel LMNA mutation in atypical Werner syndrome presenting with ischemic disease. 2009, Pubmed
Reynolds, Rapid DNA double-strand breaks resulting from processing of Cr-DNA cross-links by both MutS dimers. 2009, Pubmed
Robinson, c-Myc accelerates S-phase and requires WRN to avoid replication stress. 2009, Pubmed
Rodriguez-Lopez, Correction of proliferation and drug sensitivity defects in the progeroid Werner's Syndrome by Holliday junction resolution. 2007, Pubmed
Rodríguez-López, Characterisation of the interaction between WRN, the helicase/exonuclease defective in progeroid Werner's syndrome, and an essential replication factor, PCNA. 2003, Pubmed
Rodríguez-López, Asymmetry of DNA replication fork progression in Werner's syndrome. 2002, Pubmed
Saintigny, Homologous recombination resolution defect in werner syndrome. 2002, Pubmed
Sakamoto, Werner helicase relocates into nuclear foci in response to DNA damaging agents and co-localizes with RPA and Rad51. 2001, Pubmed
Salk, Growth characteristics of Werner syndrome cells in vitro. 1985, Pubmed
Sallmyr, Up-regulation of WRN and DNA ligase IIIalpha in chronic myeloid leukemia: consequences for the repair of DNA double-strand breaks. 2008, Pubmed , Xenbase
Sasakawa, Accumulation of FFA-1, the Xenopus homolog of Werner helicase, and DNA polymerase delta on chromatin in response to replication fork arrest. 2006, Pubmed , Xenbase
Satoh, Association between oxidative DNA damage and telomere shortening in circulating endothelial progenitor cells obtained from metabolic syndrome patients with coronary artery disease. 2008, Pubmed
Saunders, Identification and characterization of a Drosophila ortholog of WRN exonuclease that is required to maintain genome integrity. 2008, Pubmed
Schurman, Direct and indirect roles of RECQL4 in modulating base excision repair capacity. 2009, Pubmed
Sharma, Biochemical analysis of the DNA unwinding and strand annealing activities catalyzed by human RECQ1. 2005, Pubmed
Sharma, The interaction site of Flap Endonuclease-1 with WRN helicase suggests a coordination of WRN and PCNA. 2005, Pubmed
Sharma, Mechanisms of RecQ helicases in pathways of DNA metabolism and maintenance of genomic stability. 2006, Pubmed
Sharma, In vivo function of the conserved non-catalytic domain of Werner syndrome helicase in DNA replication. 2004, Pubmed
Sharma, WRN helicase and FEN-1 form a complex upon replication arrest and together process branchmigrating DNA structures associated with the replication fork. 2004, Pubmed
Sharma, Stimulation of flap endonuclease-1 by the Bloom's syndrome protein. 2004, Pubmed
Shechter, Regulation of DNA replication by ATR: signaling in response to DNA intermediates. 2004, Pubmed , Xenbase
Shen, Polymorphisms in DNA repair genes and risk of non-Hodgkin lymphoma among women in Connecticut. 2006, Pubmed
Shen, Characterization of Werner syndrome protein DNA helicase activity: directionality, substrate dependence and stimulation by replication protein A. 1998, Pubmed
Shen, Werner syndrome protein. I. DNA helicase and dna exonuclease reside on the same polypeptide. 1998, Pubmed
Shen, The N-terminal domain of the large subunit of human replication protein A binds to Werner syndrome protein and stimulates helicase activity. 2003, Pubmed
Shiloh, The ATM-mediated DNA-damage response: taking shape. 2006, Pubmed , Xenbase
Shiloh, ATM and related protein kinases: safeguarding genome integrity. 2003, Pubmed
Shiratori, WRN helicase accelerates the transcription of ribosomal RNA as a component of an RNA polymerase I-associated complex. 2002, Pubmed
Sidorova, The RecQ helicase WRN is required for normal replication fork progression after DNA damage or replication fork arrest. 2008, Pubmed
Sidorova, Roles of the Werner syndrome RecQ helicase in DNA replication. 2008, Pubmed
Siitonen, Molecular defect of RAPADILINO syndrome expands the phenotype spectrum of RECQL diseases. 2003, Pubmed
Singh, Roles of RECQ helicases in recombination based DNA repair, genomic stability and aging. 2009, Pubmed
Sobol, Requirement of mammalian DNA polymerase-beta in base-excision repair. 1996, Pubmed
Sommers, p53 modulates RPA-dependent and RPA-independent WRN helicase activity. 2005, Pubmed
Sowd, Mechanism and substrate specificity of telomeric protein POT1 stimulation of the Werner syndrome helicase. 2008, Pubmed
Spillare, p53-mediated apoptosis is attenuated in Werner syndrome cells. 1999, Pubmed
Stinco, Multiple cutaneous neoplasms in a patient with Rothmund-Thomson syndrome: case report and published work review. 2008, Pubmed
Sun, The Bloom's syndrome helicase unwinds G4 DNA. 1998, Pubmed
Suzuki, DNA helicase activity in purified human RECQL4 protein. 2009, Pubmed
Suzuki, DNA helicase activity in Werner's syndrome gene product synthesized in a baculovirus system. 1997, Pubmed
Szekely, Werner protein protects nonproliferating cells from oxidative DNA damage. 2005, Pubmed
Szekely, Werner protein recruits DNA polymerase delta to the nucleolus. 2000, Pubmed
Takeuchi, Altered frequency of initiation sites of DNA replication in Werner's syndrome cells. 1982, Pubmed
Takeuchi, Prolongation of S phase and whole cell cycle in Werner's syndrome fibroblasts. 1982, Pubmed
Thompson, Recombinational DNA repair and human disease. 2002, Pubmed
Van Maldergem, Revisiting the craniosynostosis-radial ray hypoplasia association: Baller-Gerold syndrome caused by mutations in the RECQL4 gene. 2006, Pubmed
Vennos, Rothmund-Thomson syndrome: review of the world literature. 1992, Pubmed
Von Kobbe, Werner syndrome cells escape hydrogen peroxide-induced cell proliferation arrest. 2004, Pubmed
Wai, Telomeres, telomerase, and tumorigenesis--a review. 2004, Pubmed
Wang, A polymorphism in Werner syndrome gene is associated with breast cancer susceptibility in Chinese women. 2009, Pubmed
Wang, Human Bloom protein stimulates flap endonuclease 1 activity by resolving DNA secondary structure. 2005, Pubmed
Wang, DNA topoisomerases. 1996, Pubmed
Wirtenberger, Interaction of Werner and Bloom syndrome genes with p53 in familial breast cancer. 2006, Pubmed
Wold, Replication protein A: a heterotrimeric, single-stranded DNA-binding protein required for eukaryotic DNA metabolism. 1997, Pubmed
Woods, p14 Arf promotes small ubiquitin-like modifier conjugation of Werners helicase. 2004, Pubmed
Wu, Pot1 deficiency initiates DNA damage checkpoint activation and aberrant homologous recombination at telomeres. 2006, Pubmed
Xu, Dual DNA unwinding activities of the Rothmund-Thomson syndrome protein, RECQ4. 2009, Pubmed
Xue, A minimal exonuclease domain of WRN forms a hexamer on DNA and possesses both 3'- 5' exonuclease and 5'-protruding strand endonuclease activities. 2002, Pubmed
Yan, FFA-1, a protein that promotes the formation of replication centers within nuclei. 1995, Pubmed , Xenbase
Yan, Replication focus-forming activity 1 and the Werner syndrome gene product. 1998, Pubmed , Xenbase
Yang, The processing of Holliday junctions by BLM and WRN helicases is regulated by p53. 2002, Pubmed
Yannone, Werner syndrome protein is regulated and phosphorylated by DNA-dependent protein kinase. 2001, Pubmed
Ye, Association of a polymorphic variant of the Werner helicase gene with myocardial infarction in a Japanese population. 1997, Pubmed
Zaug, Human POT1 disrupts telomeric G-quadruplexes allowing telomerase extension in vitro. 2005, Pubmed
Zheng, Novel function of the flap endonuclease 1 complex in processing stalled DNA replication forks. 2005, Pubmed
Zhou, The effect of 2-deoxy-D-glucose on Werner syndrome RecQ helicase gene. 2009, Pubmed
d'Adda di Fagagna, Functional links between telomeres and proteins of the DNA-damage response. 2004, Pubmed
d'Adda di Fagagna, A DNA damage checkpoint response in telomere-initiated senescence. 2003, Pubmed
de Lange, Shelterin: the protein complex that shapes and safeguards human telomeres. 2005, Pubmed
de Lange, Protection of mammalian telomeres. 2002, Pubmed
von Kobbe, Colocalization, physical, and functional interaction between Werner and Bloom syndrome proteins. 2002, Pubmed
von Kobbe, Central role for the Werner syndrome protein/poly(ADP-ribose) polymerase 1 complex in the poly(ADP-ribosyl)ation pathway after DNA damage. 2003, Pubmed