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    Trends Genet. 2000 May;16(5):213-20.

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

    Shen JC, Loeb LA.

    Source

    Department of Pathology, University of Washington, Seattle, Washington 98195-7705, USA. jcshen@u.wsshington.edu

    Abstract

    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.

    PMID:
    10782115
    [PubMed - indexed for MEDLINE]

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