Literature DB >> 12771022

Werner syndrome and the function of the Werner protein; what they can teach us about the molecular aging process.

Patricia L Opresko1, Wen-Hsing Cheng, Cayetano von Kobbe, Jeanine A Harrigan, Vilhelm A Bohr.   

Abstract

Werner syndrome (WS) is a hallmark premature aging disease, in which the patients appear much older than their chronological age, and exhibit many of the clinical signs and symptoms of normal aging at an early stage in life. They develop many age-associated diseases early in life including atherosclerosis, osteoporosis, cataracts and display a high incidence of cancer. WS is also marked by increased genomic instability, manifested as chromosomal alterations. Characterization and study of the Werner protein (WRN) suggests that it participates in several important DNA metabolic pathways, and that its primary function may be in DNA repair processes. Thus, the WRN protein represents an important link between defective DNA repair and the processes related to aging and cancer.

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Year:  2003        PMID: 12771022     DOI: 10.1093/carcin/bgg034

Source DB:  PubMed          Journal:  Carcinogenesis        ISSN: 0143-3334            Impact factor:   4.944


  50 in total

1.  YB-1 promotes strand separation in vitro of duplex DNA containing either mispaired bases or cisplatin modifications, exhibits endonucleolytic activities and binds several DNA repair proteins.

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2.  DNA end joining becomes less efficient and more error-prone during cellular senescence.

Authors:  Andrei Seluanov; David Mittelman; Olivia M Pereira-Smith; John H Wilson; Vera Gorbunova
Journal:  Proc Natl Acad Sci U S A       Date:  2004-04-28       Impact factor: 11.205

3.  Human cytomegalovirus UL97 kinase prevents the deposition of mutant protein aggregates in cellular models of Huntington's disease and ataxia.

Authors:  Cristy Tower; Lianwu Fu; Rachel Gill; Mark Prichard; Mathieu Lesort; Elizabeth Sztul
Journal:  Neurobiol Dis       Date:  2010-08-20       Impact factor: 5.996

Review 4.  Genomic integrity and the ageing brain.

Authors:  Hei-man Chow; Karl Herrup
Journal:  Nat Rev Neurosci       Date:  2015-10-14       Impact factor: 34.870

Review 5.  Developing master keys to brain pathology, cancer and aging from the structural biology of proteins controlling reactive oxygen species and DNA repair.

Authors:  J J P Perry; L Fan; J A Tainer
Journal:  Neuroscience       Date:  2006-12-15       Impact factor: 3.590

6.  A role for WRN in telomere-based DNA damage responses.

Authors:  Mark S Eller; Xiaodong Liao; SuiYang Liu; Kendra Hanna; Helena Bäckvall; Patricia L Opresko; Vilhelm A Bohr; Barbara A Gilchrest
Journal:  Proc Natl Acad Sci U S A       Date:  2006-10-02       Impact factor: 11.205

Review 7.  DNA damage responses in neural cells: Focus on the telomere.

Authors:  P Zhang; C Dilley; M P Mattson
Journal:  Neuroscience       Date:  2007-01-04       Impact factor: 3.590

8.  Epigenetic inactivation of the premature aging Werner syndrome gene in human cancer.

Authors:  Ruben Agrelo; Wen-Hsing Cheng; Fernando Setien; Santiago Ropero; Jesus Espada; Mario F Fraga; Michel Herranz; Maria F Paz; Montserrat Sanchez-Cespedes; Maria Jesus Artiga; David Guerrero; Antoni Castells; Cayetano von Kobbe; Vilhelm A Bohr; Manel Esteller
Journal:  Proc Natl Acad Sci U S A       Date:  2006-05-24       Impact factor: 11.205

Review 9.  The clinical characteristics of Werner syndrome: molecular and biochemical diagnosis.

Authors:  Meltem Muftuoglu; Junko Oshima; Cayetano von Kobbe; Wen-Hsing Cheng; Dru F Leistritz; Vilhelm A Bohr
Journal:  Hum Genet       Date:  2008-09-23       Impact factor: 4.132

Review 10.  DNA damage, vascular senescence and atherosclerosis.

Authors:  Maria Grazia Andreassi
Journal:  J Mol Med (Berl)       Date:  2008-06-19       Impact factor: 4.599

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