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Werner syndrome: Association of premature aging and cancer predisposition

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Werner syndrome : Association of premature aging and cancer predisposition. / Ahn, Byungchan; Stevnsner, Tinna; Bohr, Vilhelm A.

Molecular Oncology: Causes of Cancer and Targets for Treatment. red. / Edward P. Gelmann; Charles L. Sawyers ; Frank J. Rauscher, III . Cambridge University Press, 2015. s. 423-433.

Publikation: Bidrag til bog/antologi/rapport/proceedingBidrag til bog/antologiForskningpeer review

Harvard

Ahn, B, Stevnsner, T & Bohr, VA 2015, Werner syndrome: Association of premature aging and cancer predisposition. i EP Gelmann, CL Sawyers & FJ Rauscher, III (red), Molecular Oncology: Causes of Cancer and Targets for Treatment. Cambridge University Press, s. 423-433. https://doi.org/10.1017/CBO9781139046947.036

APA

Ahn, B., Stevnsner, T., & Bohr, V. A. (2015). Werner syndrome: Association of premature aging and cancer predisposition. I E. P. Gelmann, C. L. Sawyers , & F. J. Rauscher, III (red.), Molecular Oncology: Causes of Cancer and Targets for Treatment (s. 423-433). Cambridge University Press. https://doi.org/10.1017/CBO9781139046947.036

CBE

Ahn B, Stevnsner T, Bohr VA. 2015. Werner syndrome: Association of premature aging and cancer predisposition. Gelmann EP, Sawyers CL, Rauscher, III FJ, red. I Molecular Oncology: Causes of Cancer and Targets for Treatment. Cambridge University Press. s. 423-433. https://doi.org/10.1017/CBO9781139046947.036

MLA

Ahn, Byungchan, Tinna Stevnsner og Vilhelm A. Bohr "Werner syndrome: Association of premature aging and cancer predisposition"., Gelmann, Edward P. Sawyers , Charles L. Rauscher, III , Frank J. (red.). Molecular Oncology: Causes of Cancer and Targets for Treatment. Cambridge University Press. 2015, 423-433. https://doi.org/10.1017/CBO9781139046947.036

Vancouver

Ahn B, Stevnsner T, Bohr VA. Werner syndrome: Association of premature aging and cancer predisposition. I Gelmann EP, Sawyers CL, Rauscher, III FJ, red., Molecular Oncology: Causes of Cancer and Targets for Treatment. Cambridge University Press. 2015. s. 423-433 https://doi.org/10.1017/CBO9781139046947.036

Author

Ahn, Byungchan ; Stevnsner, Tinna ; Bohr, Vilhelm A. / Werner syndrome : Association of premature aging and cancer predisposition. Molecular Oncology: Causes of Cancer and Targets for Treatment. red. / Edward P. Gelmann ; Charles L. Sawyers ; Frank J. Rauscher, III . Cambridge University Press, 2015. s. 423-433

Bibtex

@inbook{dfa7fd3564d2424c84f27829e454893f,
title = "Werner syndrome: Association of premature aging and cancer predisposition",
abstract = "Introduction Werner syndrome (WS) is a rare autosomal recessive disorder in humans characterized by segmental premature aging. Somatic cells from WS individuals show low replicative capacity, hypersensitivity to DNA-damaging agents, increased genome instability, and altered telomere maintenance (1). The incidence of sarcomas is significantly higher in WS patients than in normal individuals (1). Genetic evidence indicates that WS is caused by loss of function mutations in the WRN gene, which encodes a protein that belongs to the RecQ family of DNA helicases. Polymorphisms in WRN have been demonstrated to be associated with breast cancer (2) and soft tissue sarcomas (3,4). The WRN gene has been cloned and the properties of WS cells and WRN protein have been studied extensively. Nevertheless, the pathophysiology of WS and the cellular and molecular mechanisms involved in WS pathogenesis remain poorly understood. Because WS is a progerioid disease, it is used as a model system to better understand the process of aging in humans. The exact biological role of WRN remains unknown. However, it has been proposed that it plays roles in the response to DNA damage. In particular, WRN may promote rescue of stalled replication forks and help resolve recombination intermediates in cells experiencing replicative stress. WRN also plays a role in telomere replication and alternative lengthening of telomeres. Thus, loss of function mutations in WRN could disrupt DNA replication and DNA-damage processing, resulting in accumulation of DNA double-strand breaks (DSBs) and recombination intermediates. These events could activate cell-cycle checkpoints resulting in senescence, apoptosis, or genetic instability leading to cellular transformation. These molecular endpoints could ultimately lead to features of premature aging and/or increased cancer susceptibility (Figure 35.1). This chapter discusses how WRN may contribute to genome stability and reduce cancer susceptibility.",
author = "Byungchan Ahn and Tinna Stevnsner and Bohr, {Vilhelm A.}",
year = "2015",
doi = "10.1017/CBO9781139046947.036",
language = "English",
isbn = "9780521876629",
pages = "423--433",
editor = "Gelmann, {Edward P. } and {Sawyers }, {Charles L. } and {Rauscher, III }, {Frank J. }",
booktitle = "Molecular Oncology",
publisher = "Cambridge University Press",

}

RIS

TY - CHAP

T1 - Werner syndrome

T2 - Association of premature aging and cancer predisposition

AU - Ahn, Byungchan

AU - Stevnsner, Tinna

AU - Bohr, Vilhelm A.

PY - 2015

Y1 - 2015

N2 - Introduction Werner syndrome (WS) is a rare autosomal recessive disorder in humans characterized by segmental premature aging. Somatic cells from WS individuals show low replicative capacity, hypersensitivity to DNA-damaging agents, increased genome instability, and altered telomere maintenance (1). The incidence of sarcomas is significantly higher in WS patients than in normal individuals (1). Genetic evidence indicates that WS is caused by loss of function mutations in the WRN gene, which encodes a protein that belongs to the RecQ family of DNA helicases. Polymorphisms in WRN have been demonstrated to be associated with breast cancer (2) and soft tissue sarcomas (3,4). The WRN gene has been cloned and the properties of WS cells and WRN protein have been studied extensively. Nevertheless, the pathophysiology of WS and the cellular and molecular mechanisms involved in WS pathogenesis remain poorly understood. Because WS is a progerioid disease, it is used as a model system to better understand the process of aging in humans. The exact biological role of WRN remains unknown. However, it has been proposed that it plays roles in the response to DNA damage. In particular, WRN may promote rescue of stalled replication forks and help resolve recombination intermediates in cells experiencing replicative stress. WRN also plays a role in telomere replication and alternative lengthening of telomeres. Thus, loss of function mutations in WRN could disrupt DNA replication and DNA-damage processing, resulting in accumulation of DNA double-strand breaks (DSBs) and recombination intermediates. These events could activate cell-cycle checkpoints resulting in senescence, apoptosis, or genetic instability leading to cellular transformation. These molecular endpoints could ultimately lead to features of premature aging and/or increased cancer susceptibility (Figure 35.1). This chapter discusses how WRN may contribute to genome stability and reduce cancer susceptibility.

AB - Introduction Werner syndrome (WS) is a rare autosomal recessive disorder in humans characterized by segmental premature aging. Somatic cells from WS individuals show low replicative capacity, hypersensitivity to DNA-damaging agents, increased genome instability, and altered telomere maintenance (1). The incidence of sarcomas is significantly higher in WS patients than in normal individuals (1). Genetic evidence indicates that WS is caused by loss of function mutations in the WRN gene, which encodes a protein that belongs to the RecQ family of DNA helicases. Polymorphisms in WRN have been demonstrated to be associated with breast cancer (2) and soft tissue sarcomas (3,4). The WRN gene has been cloned and the properties of WS cells and WRN protein have been studied extensively. Nevertheless, the pathophysiology of WS and the cellular and molecular mechanisms involved in WS pathogenesis remain poorly understood. Because WS is a progerioid disease, it is used as a model system to better understand the process of aging in humans. The exact biological role of WRN remains unknown. However, it has been proposed that it plays roles in the response to DNA damage. In particular, WRN may promote rescue of stalled replication forks and help resolve recombination intermediates in cells experiencing replicative stress. WRN also plays a role in telomere replication and alternative lengthening of telomeres. Thus, loss of function mutations in WRN could disrupt DNA replication and DNA-damage processing, resulting in accumulation of DNA double-strand breaks (DSBs) and recombination intermediates. These events could activate cell-cycle checkpoints resulting in senescence, apoptosis, or genetic instability leading to cellular transformation. These molecular endpoints could ultimately lead to features of premature aging and/or increased cancer susceptibility (Figure 35.1). This chapter discusses how WRN may contribute to genome stability and reduce cancer susceptibility.

UR - http://www.scopus.com/inward/record.url?scp=84954119870&partnerID=8YFLogxK

U2 - 10.1017/CBO9781139046947.036

DO - 10.1017/CBO9781139046947.036

M3 - Book chapter

AN - SCOPUS:84954119870

SN - 9780521876629

SP - 423

EP - 433

BT - Molecular Oncology

A2 - Gelmann, Edward P.

A2 - Sawyers , Charles L.

A2 - Rauscher, III , Frank J.

PB - Cambridge University Press

ER -