Recapitulation of premature ageing with iPSCs from Hutchinson–Gilford progeria syndrome

Guang-Hui Liu, Basam Z. Barkho, Sergio Ruiz, Dinh Diep, Jing Qu, Sheng-Lian Yang, Athanasia D. Panopoulos, Keiichiro Suzuki, Leo Kurian, Christopher Walsh, James Thompson, Stephanie Boue, Ho Lim Fung, Ignacio Sancho-Martinez, Kun Zhang, John Yates Yates and Juan Carlos Izpisua Belmonte ()
Additional contact information
Guang-Hui Liu: Gene Expression Laboratory, Salk Institute for Biological Studies
Basam Z. Barkho: Gene Expression Laboratory, Salk Institute for Biological Studies
Sergio Ruiz: Gene Expression Laboratory, Salk Institute for Biological Studies
Dinh Diep: University of California at San Diego
Jing Qu: Gene Expression Laboratory, Salk Institute for Biological Studies
Sheng-Lian Yang: Gene Expression Laboratory, Salk Institute for Biological Studies
Athanasia D. Panopoulos: Gene Expression Laboratory, Salk Institute for Biological Studies
Keiichiro Suzuki: Gene Expression Laboratory, Salk Institute for Biological Studies
Leo Kurian: Gene Expression Laboratory, Salk Institute for Biological Studies
Christopher Walsh: Gene Expression Laboratory, Salk Institute for Biological Studies
James Thompson: Scripps Research Institute
Stephanie Boue: Center for Regenerative Medicine in Barcelona, Dr. Aiguader 88
Ho Lim Fung: University of California at San Diego
Ignacio Sancho-Martinez: Gene Expression Laboratory, Salk Institute for Biological Studies
Kun Zhang: University of California at San Diego
John Yates Yates: Scripps Research Institute
Juan Carlos Izpisua Belmonte: Gene Expression Laboratory, Salk Institute for Biological Studies

Nature, 2011, vol. 472, issue 7342, 221-225

Abstract: An in vitro model of premature ageing The premature ageing disorder Hutchinson–Gilford progeria syndrome (HGPS) is a rare genetic condition characterized by a rapid onset of signs associated with normal ageing, such as atherosclerosis and the degeneration of vascular smooth-muscle cells. Liu et al. report that the altered structure of the nuclear envelope and epigenetic modifications that accumulate during physiological ageing or under specific disease conditions can be restored to normalcy by reprogramming somatic cell lines established with fibroblasts from patients with HGPS as induced pluripotent stem (iPS) cells. Directed differentiation of the resulting iPS cells as vascular smooth-muscle cells then leads to the appearance of the premature senescence phenotypes associated with vascular ageing. This HGPS iPS cell model provides a way to study the mechanisms regulating premature and normal ageing in vitro.

Date: 2011
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DOI: 10.1038/nature09879

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