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

Author

Listed:
  • 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 III

    (Scripps Research Institute)

  • Juan Carlos Izpisua Belmonte

    (Gene Expression Laboratory, Salk Institute for Biological Studies
    Center for Regenerative Medicine in Barcelona, Dr. Aiguader 88)

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.

Suggested Citation

  • 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 & , 2011. "Recapitulation of premature ageing with iPSCs from Hutchinson–Gilford progeria syndrome," Nature, Nature, vol. 472(7342), pages 221-225, April.
    • Handle: RePEc:nat:nature:v:472:y:2011:i:7342:d:10.1038_nature09879
    DOI: 10.1038/nature09879
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    Cited by:

    1. Baolei Yuan & Xuan Zhou & Keiichiro Suzuki & Gerardo Ramos-Mandujano & Mengge Wang & Muhammad Tehseen & Lorena V. Cortés-Medina & James J. Moresco & Sarah Dunn & Reyna Hernandez-Benitez & Tomoaki Hish, 2022. "Wiskott-Aldrich syndrome protein forms nuclear condensates and regulates alternative splicing," Nature Communications, Nature, vol. 13(1), pages 1-20, December.
    2. Jorge Mata-Garrido & Yao Xiang & Yunhua Chang-Marchand & Caroline Reisacher & Elisabeth Ageron & Ida Chiara Guerrera & Iñigo Casafont & Aurelia Bruneau & Claire Cherbuy & Xavier Treton & Anne Dumay & , 2022. "The Heterochromatin protein 1 is a regulator in RNA splicing precision deficient in ulcerative colitis," Nature Communications, Nature, vol. 13(1), pages 1-16, December.
    3. Patricia R. Pitrez & Luis M. Monteiro & Oliver Borgogno & Xavier Nissan & Jerome Mertens & Lino Ferreira, 2024. "Cellular reprogramming as a tool to model human aging in a dish," Nature Communications, Nature, vol. 15(1), pages 1-11, December.

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