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Telomere shortening and loss of self-renewal in dyskeratosis congenita induced pluripotent stem cells

Author

Listed:
  • Luis F. Z. Batista

    (Stanford University School of Medicine)

  • Matthew F. Pech

    (Stanford University School of Medicine
    Cancer Biology Program, Stanford University School of Medicine)

  • Franklin L. Zhong

    (Stanford University School of Medicine
    Cancer Biology Program, Stanford University School of Medicine)

  • Ha Nam Nguyen

    (Institute for Stem Cell Biology & Regenerative Medicine, Stanford University School of Medicine)

  • Kathleen T. Xie

    (Stanford University School of Medicine)

  • Arthur J. Zaug

    (Howard Hughes Medical Institute, University of Colorado)

  • Sharon M. Crary

    (Howard Hughes Medical Institute, University of Colorado)

  • Jinkuk Choi

    (Stanford University School of Medicine
    Cancer Biology Program, Stanford University School of Medicine)

  • Vittorio Sebastiano

    (Institute for Stem Cell Biology & Regenerative Medicine, Stanford University School of Medicine
    Stanford University School of Medicine)

  • Athena Cherry

    (Stanford University School of Medicine)

  • Neelam Giri

    (Clinical Genetics Branch, National Cancer Institute, National Institutes of Health)

  • Marius Wernig

    (Institute for Stem Cell Biology & Regenerative Medicine, Stanford University School of Medicine
    Stanford University School of Medicine)

  • Blanche P. Alter

    (Clinical Genetics Branch, National Cancer Institute, National Institutes of Health)

  • Thomas R. Cech

    (Howard Hughes Medical Institute, University of Colorado)

  • Sharon A. Savage

    (Clinical Genetics Branch, National Cancer Institute, National Institutes of Health)

  • Renee A. Reijo Pera

    (Cancer Biology Program, Stanford University School of Medicine
    Institute for Stem Cell Biology & Regenerative Medicine, Stanford University School of Medicine)

  • Steven E. Artandi

    (Stanford University School of Medicine
    Cancer Biology Program, Stanford University School of Medicine
    The Glenn Laboratories for the Biology of Aging, Stanford University School of Medicine)

Abstract

A novel iPS cell disease model Dyskeratosis congenita, a disease characterized by defective maintenance of blood, pulmonary and epidermal tissues, is caused by defects in genes required for telomere homeostasis. Short telomeres are thought to cause signs of the disease in mouse models by inducing senescence and cell death responses that impair tissue stem-cell function. Batista et al. generated induced pluripotent stem (iPS) cells from dyskeratosis congenita patients. Previously reported iPS-cell-based disease models have required iPS cells to be differentiated to a terminal cell type before cellular defects emerge. In this instance, many features of the human stem-cell disease are found in patient-derived iPS cells, providing a model that is not dependent on cell differentiation to study disease mechanisms or to identify potential therapeutics.

Suggested Citation

  • Luis F. Z. Batista & Matthew F. Pech & Franklin L. Zhong & Ha Nam Nguyen & Kathleen T. Xie & Arthur J. Zaug & Sharon M. Crary & Jinkuk Choi & Vittorio Sebastiano & Athena Cherry & Neelam Giri & Marius, 2011. "Telomere shortening and loss of self-renewal in dyskeratosis congenita induced pluripotent stem cells," Nature, Nature, vol. 474(7351), pages 399-402, June.
    • Handle: RePEc:nat:nature:v:474:y:2011:i:7351:d:10.1038_nature10084
    DOI: 10.1038/nature10084
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