The Werner syndrome gene: the molecular basis of RecQ helicase-deficiency diseases |
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Authors: | Shen J C Loeb L A |
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Affiliation: | Department of Pathology, University of Washington, Seattle, Washington 98195-7705, USA. jcshen@u.wsshington.edu |
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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. |
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