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Telomere elongation in induced pluripotent stem cells from dyskeratosis congenita patients

Author

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  • Suneet Agarwal

    (Children’s Hospital Boston; Pediatric Oncology, Brigham and Women’s Hospital; Harvard Stem Cell Institute; Manton Center for Orphan Disease Research; Boston, Massachusetts 02115, USA
    Pediatric Oncology, Dana Farber Cancer Institute)

  • Yuin-Han Loh

    (Children’s Hospital Boston; Pediatric Oncology, Brigham and Women’s Hospital; Harvard Stem Cell Institute; Manton Center for Orphan Disease Research; Boston, Massachusetts 02115, USA
    Pediatric Oncology, Dana Farber Cancer Institute)

  • Erin M. McLoughlin

    (Children’s Hospital Boston; Pediatric Oncology, Brigham and Women’s Hospital; Harvard Stem Cell Institute; Manton Center for Orphan Disease Research; Boston, Massachusetts 02115, USA
    Pediatric Oncology, Dana Farber Cancer Institute)

  • Junjiu Huang

    (MDC 3125, University of South Florida College of Medicine, 12901 Bruce B. Downs Boulevard, Tampa, Florida 33612, USA
    State Key Laboratory of Biocontrol, School of Life Sciences, Sun Yat-sen University, Guangzhou, 510275, China)

  • In-Hyun Park

    (Children’s Hospital Boston; Pediatric Oncology, Brigham and Women’s Hospital; Harvard Stem Cell Institute; Manton Center for Orphan Disease Research; Boston, Massachusetts 02115, USA
    Pediatric Oncology, Dana Farber Cancer Institute)

  • Justine D. Miller

    (Children’s Hospital Boston; Pediatric Oncology, Brigham and Women’s Hospital; Harvard Stem Cell Institute; Manton Center for Orphan Disease Research; Boston, Massachusetts 02115, USA
    Pediatric Oncology, Dana Farber Cancer Institute)

  • Hongguang Huo

    (Children’s Hospital Boston; Pediatric Oncology, Brigham and Women’s Hospital; Harvard Stem Cell Institute; Manton Center for Orphan Disease Research; Boston, Massachusetts 02115, USA
    Pediatric Oncology, Dana Farber Cancer Institute)

  • Maja Okuka

    (MDC 3125, University of South Florida College of Medicine, 12901 Bruce B. Downs Boulevard, Tampa, Florida 33612, USA)

  • Rosana Maria dos Reis

    (MDC 3125, University of South Florida College of Medicine, 12901 Bruce B. Downs Boulevard, Tampa, Florida 33612, USA)

  • Sabine Loewer

    (Children’s Hospital Boston; Pediatric Oncology, Brigham and Women’s Hospital; Harvard Stem Cell Institute; Manton Center for Orphan Disease Research; Boston, Massachusetts 02115, USA
    Pediatric Oncology, Dana Farber Cancer Institute)

  • Huck-Hui Ng

    (Gene Regulatory Laboratory, National University of Singapore; 60 Biopolis Street, #02-01, Genome, Singapore 138672
    National University of Singapore)

  • David L. Keefe

    (MDC 3125, University of South Florida College of Medicine, 12901 Bruce B. Downs Boulevard, Tampa, Florida 33612, USA)

  • Frederick D. Goldman

    (The Children’s Hospital of Alabama, 1600 7th Avenue South, ACC 512, Birmingham, Alabama 35233, USA)

  • Aloysius J. Klingelhutz

    (University of Iowa, 2202 MERF, 375 Newton Road, Iowa City, Iowa 52242, USA)

  • Lin Liu

    (MDC 3125, University of South Florida College of Medicine, 12901 Bruce B. Downs Boulevard, Tampa, Florida 33612, USA
    College of Life Sciences, Nankai University)

  • George Q. Daley

    (Children’s Hospital Boston; Pediatric Oncology, Brigham and Women’s Hospital; Harvard Stem Cell Institute; Manton Center for Orphan Disease Research; Boston, Massachusetts 02115, USA
    Pediatric Oncology, Dana Farber Cancer Institute
    Howard Hughes Medical Institute, Children’s Hospital Boston, Karp Family Research Building 07214, 1 Blackfan Circle, Boston, Massachusetts 02115, USA)

Abstract

Restoring telomere elongation in dyskeratosis congenita Patients with dyskeratosis congenita, a disorder of telomere maintenance, suffer degeneration of multiple tissues. Agarwal et al. use iPS (induced pluripotent stem) cell technology to study the mechanisms underlying the disease in humans, and in doing so they discover that reprogramming restores telomere elongation in dyskeratosis congenita cells despite genetic lesions affecting telomerase. The reprogrammed dyskeratosis congenita cells were able to overcome a critical limitation in telomerase RNA component (TERC) levels to restore telomere maintenance and self-renewal, and multiple telomerase components are targeted by pluripotency-associated transcription factors. Strategies designed to increase TERC expression may therefore be therapeutically beneficial in dyskeratosis congenita patients.

Suggested Citation

  • Suneet Agarwal & Yuin-Han Loh & Erin M. McLoughlin & Junjiu Huang & In-Hyun Park & Justine D. Miller & Hongguang Huo & Maja Okuka & Rosana Maria dos Reis & Sabine Loewer & Huck-Hui Ng & David L. Keefe, 2010. "Telomere elongation in induced pluripotent stem cells from dyskeratosis congenita patients," Nature, Nature, vol. 464(7286), pages 292-296, March.
    • Handle: RePEc:nat:nature:v:464:y:2010:i:7286:d:10.1038_nature08792
    DOI: 10.1038/nature08792
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    Cited by:

    1. Tobias T. Schmidt & Carly Tyer & Preeyesh Rughani & Candy Haggblom & Jeffrey R. Jones & Xiaoguang Dai & Kelly A. Frazer & Fred H. Gage & Sissel Juul & Scott Hickey & Jan Karlseder, 2024. "High resolution long-read telomere sequencing reveals dynamic mechanisms in aging and cancer," Nature Communications, Nature, vol. 15(1), pages 1-11, December.

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