A mouse model of Werner Syndrome: what can it tell us about aging and cancer?

The molecular mechanisms involved in mammalian aging and the consequent organ dysfunction/degeneration pathologies are not well understood. Studies of progeroid syndromes such as Werner Syndrome have advanced our understanding of how certain genetic pathways can influence the aging process on both cellular and molecular levels. In addition, improper maintenance of telomere length and the consequent cellular responses to dysfunctional telomeres have been proposed to promote replicative senescence that impact. upon the onset of premature aging and cancer. Recent studies of the telomerase-Werner double null mouse link telomere dysfunction to accelerated aging and tumorigenesis in the setting of Werner deficiency. This mouse model thus provides a unique genetic platform to explore molecular mechanisms by which telomere dysfunction and loss of WRN gene function leads to the onset of premature aging and cancer. (c) 2004 Elsevier Ltd. All rights reserved.