Sci. Aging Knowl. Environ., 31 March 2004
Vol. 2004, Issue 13, p. re3
[DOI: 10.1126/sageke.2004.13.re3]

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Werner Syndrome Protein--Unwinding Function to Explain Disease

Raymond J. Monnat Jr., and Yannick Saintigny

Raymond J. Monnat Jr. is in the Departments of Pathology and Genome Sciences at the University of Washington, Seattle, WA 98195, USA. Yannick Saintigny is at the Laboratoire d'Etude de la Recombinaison, Département de Radiobiologie et Radiopathologie, Commissariat à l'Energie Atomique, 92265 Fontenay aux Roses Cedex, France. E-mail: monnat{at}u.washington.edu (R.J.M.)

http://sageke.sciencemag.org/cgi/content/full/2004/13/re3

Key Words: Werner syndrome • Bloom syndrome • RecQ DNA helicase • homologous recombination • nonhomologous DNA end joining • DNA repair

Abstract: Werner syndrome (WS) is one of three heritable human genetic instability/cancer predisposition syndromes that result from mutations in a member of the gene family encoding human RecQ helicases. Cellular defects are a prominent part of the WS phenotype. Here we review recent work to identify in vivo functions of the WS protein and discuss how loss of function leads to cellular defects. These new results provide clues to the origin of cell lineage-specific defects in WS patients and suggest a broader role for Werner protein function in determining disease risk in the general population.

Citation: R. J. Monnat, Y. Saintigny, Werner Syndrome Protein--Unwinding Function to Explain Disease. Sci. Aging Knowl. Environ. 2004 (13), re3 (2004).

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