The Werner syndrome gene: the molecular basis of RecQ helicase-deficiency diseases

Trends Genet. 2000 May;16(5):213-20. doi: 10.1016/s0168-9525(99)01970-8.


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.

Publication types

  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Adenosine Triphosphatases / genetics
  • Animals
  • DNA Helicases / genetics*
  • DNA Helicases / metabolism*
  • DNA Repair
  • DNA Replication
  • DNA-Binding Proteins / metabolism
  • Disease Models, Animal
  • Exodeoxyribonucleases
  • Humans
  • Mice
  • RecQ Helicases
  • Recombination, Genetic
  • Replication Protein A
  • Tumor Suppressor Protein p53 / genetics
  • Tumor Suppressor Protein p53 / metabolism
  • Werner Syndrome / genetics*
  • Werner Syndrome Helicase


  • DNA-Binding Proteins
  • RPA1 protein, human
  • Replication Protein A
  • Rpa1 protein, mouse
  • Tumor Suppressor Protein p53
  • Exodeoxyribonucleases
  • Adenosine Triphosphatases
  • RECQL protein, human
  • DNA Helicases
  • RecQ Helicases
  • WRN protein, human
  • Werner Syndrome Helicase