Abstract
This chapter reviews clinical and basic science aspects of Werner syndrome (WS), a heritable human disease that displays features suggestive of premature aging. The resemblance of changes in WS to accelerated normal aging has long suggested WS may be a useful model in which to study the biology of aging and to identify mechanisms responsible for age-associated diseases including atherosclerotic cardiovascular disease, neoplasia, diabetes mellitus, and osteoporosis. In this chapter we describe WS as a clinical disease entity; outline diagnostic criteria for WS; discuss the relationship of WS to normal aging; summarize our current understanding of the WRN genetic variation and in vivo functions of the WS protein; and discuss how loss of function may promote disease pathogenesis. We also review the more promising cellular and animal models of WS; discuss potential therapeutic strategies in WS; and conclude with a selection of useful resources to aid those interested in learning more about this fascinating and instructive human progeroid syndrome.
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