Literature DB >> 15916783

Deficient DNA repair in the human progeroid disorder, Werner syndrome.

Vilhelm A Bohr1.   

Abstract

The study of how DNA repair mechanisms change with aging is central to our understanding of the aging process. Here, I review the molecular functions of a key aging protein, Werner protein (WRN), which is deficient in the premature aging disorder, Werner syndrome (WS). This protein plays a significant role in DNA repair, particularly in base excision repair and in recombination. WRN may be a key regulatory factor in these processes and may also play a role in coordinating them. WRN belongs to the RecQ helicase family of proteins, often referred to as the guardians of the genome. These proteins appear to integrate with the more classic DNA repair pathways and proteins.

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Year:  2005        PMID: 15916783     DOI: 10.1016/j.mrfmmm.2005.03.021

Source DB:  PubMed          Journal:  Mutat Res        ISSN: 0027-5107            Impact factor:   2.433


  22 in total

Review 1.  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

Review 2.  Laminopathies: multiple disorders arising from defects in nuclear architecture.

Authors:  Veena K Parnaik; Kaliyaperumal Manju
Journal:  J Biosci       Date:  2006-09       Impact factor: 1.826

Review 3.  RecQ helicases: guardian angels of the DNA replication fork.

Authors:  Csanád Z Bachrati; Ian D Hickson
Journal:  Chromosoma       Date:  2008-01-11       Impact factor: 4.316

Review 4.  Replicative aging in yeast: the means to the end.

Authors:  K A Steinkraus; M Kaeberlein; B K Kennedy
Journal:  Annu Rev Cell Dev Biol       Date:  2008       Impact factor: 13.827

Review 5.  DNA polymerase epsilon: a polymerase of unusual size (and complexity).

Authors:  Zachary F Pursell; Thomas A Kunkel
Journal:  Prog Nucleic Acid Res Mol Biol       Date:  2008

6.  WRN protects against topo I but not topo II inhibitors by preventing DNA break formation.

Authors:  Markus Christmann; Maja T Tomicic; Christopher Gestrich; Wynand P Roos; Vilhelm A Bohr; Bernd Kaina
Journal:  DNA Repair (Amst)       Date:  2008-10-15

Review 7.  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

8.  Early embryonic lethality of mice lacking the essential protein SNEV.

Authors:  Klaus Fortschegger; Bettina Wagner; Regina Voglauer; Hermann Katinger; Maria Sibilia; Johannes Grillari
Journal:  Mol Cell Biol       Date:  2007-02-05       Impact factor: 4.272

9.  Cigarette smoke induces cellular senescence via Werner's syndrome protein down-regulation.

Authors:  Toru Nyunoya; Martha M Monick; Aloysius L Klingelhutz; Heather Glaser; Jeffrey R Cagley; Charles O Brown; Eiyu Matsumoto; Nukhet Aykin-Burns; Douglas R Spitz; Junko Oshima; Gary W Hunninghake
Journal:  Am J Respir Crit Care Med       Date:  2008-11-14       Impact factor: 21.405

Review 10.  From old organisms to new molecules: integrative biology and therapeutic targets in accelerated human ageing.

Authors:  L S Cox; R G A Faragher
Journal:  Cell Mol Life Sci       Date:  2007-10       Impact factor: 9.261

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