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Letter

Nature Genetics 36, 877–882 (1 August 2004) | doi:10.1038/ng1389

Essential role of limiting telomeres in the pathogenesis of Werner syndrome

Sandy Chang , Asha S Multani , Noelia G Cabrera , Maria L Naylor , Purnima Laud , David Lombard , Sen Pathak , Leonard Guarente & Ronald A DePinho

Mutational inactivation of the gene WRN causes Werner syndrome, an autosomal recessive disease characterized by premature aging, elevated genomic instability and increased cancer incidence. The capacity of enforced telomerase expression to rescue premature senescence of cultured cells from individuals with Werner syndrome and the lack of a disease phenotype in Wrn-deficient mice with long telomeres implicate telomere attrition in the pathogenesis of Werner syndrome.