Speeding up the clock: The past, present and future of progeria. (21st December 2015)

• Record Type: Journal Article
• Authors: Swahari, Vijay; Nakamura, Ayumi
• Is Part Of: Development growth and differentiation. Volume 58:Number 1(2016)
• Page Start: 116
• Page End: 130
• Publication Date: 2015-12-21
• Subjects: Hutchinson‐Gilford Progeria Syndrome -- mouse models -- premature aging -- progeria -- senescence; Embryology -- Periodicals; Developmental biology -- Periodicals; Growth -- Periodicals; 574.3
• DOI: 10.1111/dgd.12251
• Languages: English
• ISSNs: 0012-1592

Abstract

Abstract : Progeria is a devastating disorder in which patients exhibit signs of premature aging. The most well‐known progeroid syndromes include Hutchinson‐Gilford Progeria Syndrome (HGPS) and Werner Syndrome (WS). While HGPS and WS are rare, they often result in severe age‐associated complications starting in the early developmental period or after the pubertal growth spurt during adolescence, respectively. In addition, patients with HGPS ultimately die of diseases normally seen in the elderly population, with stroke and myocardial infarction as the leading causes of death. Many WS patients develop similar cardiovascular complications but also have an increased predisposition to developing multiple rare malignancies. These premature aging disorders, as well as animal and cell culture models that recapitulate these diseases, have provided insight into the genetics and cellular pathways that underlie these human conditions and have also uncovered possible mechanisms behind normal aging. Here we discuss the history, the types of progeria, and the various pathophysiological mechanisms that drive these diseases. We also address recent medical advances and treatment modalities for patients with progeria. Abstract : Patients afflicted with progeroid syndromes such as Hutchinson‐Gilford Progeria Syndrome and Werner Syndrome exhibit features of premature aging and die of age‐associated diseases often seen in the aging population. This review covers the genetic basis of progeria as well as the animal and cell culture models that have been instrumental in the development of treatment strategies for patients with these progeroid syndromes.