Molecular Mechanisms of Werner's Syndrome
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Edited By:Michel LebelUniversité Laval ISBN: 978-0-306-48233-5 Published: 2004-07-27 This book may be purchased as an eBook (pdf) for $99, or individual chapters (pdf) may be purchased from the list below for $19. |
Chapters available from this book
Replicative Senescence, Telomeres and Werner’s Syndrome
Richard G.A. Faragher
Werner’s syndrome (WS) is studied as a model of accelerated aging and results from mutations in a recQ helicase (WRN). WS fibroblasts show a mutator phenotype (producing large DNA deletions), replication fork stalling, increased rates of mean telomeric loss and accelerated replicative senescence....
Proposed Biological Functions for the Werner Syndrome Protein in DNA Metabolism
Patricia L. Opresko, Jeanine A. Harrigan, Wen-Hsing Cheng, Robert M. Brosh, Jr. and Vilhelm A. Bohr
Werner syndrome is a premature aging disease that is characterized by genomic instability. The gene defective in Werner syndrome encodes a protein with helicase and exonuclease activities. This chapter focuses on the proposed roles of the Werner syndrome protein in various aspects of DNA metaboli...
Proteins That Interact with the Werner Syndrome Gene Product
Dana Branzei and Takemi Enomoto
Werner syndrome is an autosomal recessive disorder characterized by premature onset of age-related diseases, increased cancer incidence, and genomic instability. Biochemical characterization has shown WRN protein to have helicase and exonuclease activities of 3'-5' polarity, and to have an associ...
Clinical Aspects of Werner’s Syndrome: Its Natural History and the Genetics of the Disease
Makoto Goto
Werner’s syndrome, caused by a mutation in the WRN (or RecQ3) helicase gene, shows a variety of clinical and biochemical-aging phenotypes at an early stage of life followed by death at an average age of 46 years old. It has been nominated as a top ranking progeroid syndrome. Consequently, analyse...
Yeast RecQ Helicases: Clues to DNA Repair, Genome Stability and Aging
Rozalyn M. Anderson and David A. Sinclair
The budding yeast Saccharomyces cerevisiae has been used as model for a wide range of cellular processes, including those related to the RecQ-associated progeroid disease, Werner’s syndrome (WS). Investigations of RecQ function in these lower eukaryotes have produced a large body of data that is ...
Biochemical Characterization of the Werner Syndrome DNA Helicase—Exonuclease
Michael Fry
The positional cloning in 1996 of WRN, the human gene defective in Werner syndrome (WS), launched an explosive experimental activity that culminated in the expression, purification and comprehensive characterization of its product protein – the WRN DNA helicase-exonuclease. Being a member of the Rec...
Biochemical Roles of RecQ Helicases
Payam Mohaghegh and Ian D. Hickson
The RecQ family of DNA helicases appears to influence DNA repair, replication and/or homologous recombination pathways. In humans, a defect in the RecQ family helicases encoded by the BLM, WRN and RECQ4 genes gives rise to Bloom’s, Werner’s and Rothmund-Thomson syndromes, respectively. These d...
Sensitivity of Werner's Syndrome Cells to DNA Damaging Agents: Insights into the Biological Functions of the Werner Protein
Adayabalam S. Balajee and Fabrizio Palitti
Werner’s syndrome (WS) is a human autosomal recessive disorder characterized by many symptoms of accelerated aging. The gene responsible for Werner’s syndrome (WRN) has been cloned and the protein has been biochemically characterized as a helicase/exonuclease. Determination of the preferred DNA subs...
Potential Function of the Werner's Syndrome Homologue in the African Clawed Frog and the Mouse
Michel Lebel and Philip Leder
After the discovery of the gene responsible for WS in the human, genes with high homology to WRN were found in the mouse Mus musculus and the African clawed frog Xenopus laevis genome. These laboratory animals brought new experimental approaches in which to study and understand the potential...
