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Titre du Document
The Werner syndrome gene : the molecular basis of RecQ helicase-deficiency diseases
Auteur(s)
SHEN J.-C. ; LOEB L. A.
Résumé
Werner syndrome (WS) is an autosomal recessive genetic disorder that is manifested by genetic instability and premature onset of age-related diseases, including atherosclerosis and cancer. The gene that is mutated in WS cells (WRN) has been identified recently. Characterizations of the WRN gene product indicate that WRN encodes both a 3'→5' DNA helicase, belonging to the Escherichia coli RecQ helicase family, and a 3'→5' DNA exonuclease. Studies to define the molecular mechanism of WRN-DNA transactions are currently underway in many laboratories. Preliminary results indicate that WRN functions as a key factor in resolving aberrant DNA structures that arise from DNA metabolic processes such as replication, recombination and/or repair, to preserve the genetic integrity in cells.
Editeur
Elsevier Science
Identifiant
PMID : 10782115 ISSN : 0168-9525
Source
Trends in genetics (Regular ed.)
A. 2000, vol. 16, n° 5, pp. 213-220 [bibl. : 73 ref.]
Langue
Anglais
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