Journal Article

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

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

in Carcinogenesis

Volume 24, issue 5, pages 791-802
Published in print May 2003 | ISSN: 0143-3334
Published online May 2003 | e-ISSN: 1460-2180 | DOI: http://dx.doi.org/10.1093/carcin/bgg034
Werner syndrome and the function of the Werner protein; what they can teach us about the molecular aging process.

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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.

Keywords: AA-PML, ALT-associated PML bodies; BER, base excision repair; BS, Bloom syndrome; CPT, camptothecin; dRP, 5′-deoxyribose 5-phosphate; D-loop, displacement loop; DSB, double strand break; HR, homologous recombination; IR, ionizing radiation; NHEJ, non-homologous end joining; 4-NQO, 4-nitroquinoline 1-oxide; NTS, nucleolar targeting sequence; PCNA, proliferating cell nuclear antigen; topo I, topoisomerase I; UV, ultraviolet; WRN, Werner protein; WS, Werner syndrome

Journal Article.  10480 words.  Illustrated.

Subjects: Clinical Cytogenetics and Molecular Genetics

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