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

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 Wernig, Blanche P. Alter, Thomas R. Cech, Sharon A. Savage, Renee A. Reijo Pera and Steven E. Artandi ()
Additional contact information
Luis F. Z. Batista: Stanford University School of Medicine
Matthew F. Pech: Stanford University School of Medicine
Franklin L. Zhong: 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
Vittorio Sebastiano: Institute for Stem Cell Biology & Regenerative 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
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
Steven E. Artandi: Stanford University School of Medicine

Nature, 2011, vol. 474, issue 7351, 399-402

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.

Date: 2011
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DOI: 10.1038/nature10084

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