The Werner syndrome gene - the molecular basis of RecQ helicase-deficiencydiseases

Werner syndrome (WS) is an autosomal recessive genetic disorder that is man ifested 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 prod uct indicate that WRN encodes both a 3'-->5' DNA helicase, belonging to the Escherichia coli RecQ helicase family, and a 3'-->5' DNA exonuclease. Stud ies to define the molecular mechanism of WRN-DNA transactions are currently underway in many laboratories. Preliminary results indicate that WRN funct ions as a key factor in resolving aberrant DNA structures that arise from D NA metabolic processes such as replication, recombination and/or repair, to preserve the genetic integrity in cells.