The Werner syndrome gene: the molecular basis of RecQ helicase-deficiency diseases.
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 Escherichiacoli 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.
Shen
J C
JC
Department of Pathology, University of Washington, Seattle, Washington 98195-7705, USA. jcshen@u.wsshington.edu
Loeb
L A
LA
D000251:Adenosine Triphosphatases / Q000235:genetics; D000818:Animals; D004265:DNA Helicases / Q000235:genetics* / Q000378:metabolism*; D004260:DNA Repair; D004261:DNA Replication; D004268:DNA-Binding Proteins / Q000378:metabolism; D004195:Disease Models, Animal; D005090:Exodeoxyribonucleases; D006801:Humans; D051379:Mice; D053484:RecQ Helicases; D011995:Recombination, Genetic; D051716:Replication Protein A; D016159:Tumor Suppressor Protein p53 / Q000235:genetics / Q000378:metabolism; D014898:Werner Syndrome / Q000235:genetics*; D000071657:Werner Syndrome Helicase
D004268:DNA-Binding Proteins; C497167:RPA1 protein, human; D051716:Replication Protein A; C497168:Rpa1 protein, mouse; D016159:Tumor Suppressor Protein p53; D005090:Exodeoxyribonucleases; D000251:Adenosine Triphosphatases; C506725:RECQL protein, human; D004265:DNA Helicases; D053484:RecQ Helicases; C098785:WRN protein, human; D000071657:Werner Syndrome Helicase