Telomere elongation in induced pluripotent stem cells from dyskeratosis congenita patients

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, Frederick D. Goldman, Aloysius J. Klingelhutz, Lin Liu and George Q. Daley ()
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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
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
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
Junjiu Huang: MDC 3125, University of South Florida College of Medicine, 12901 Bruce B. Downs Boulevard, Tampa, Florida 33612, USA
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
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
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
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
Huck-Hui Ng: Gene Regulatory Laboratory, National University of Singapore; 60 Biopolis Street, #02-01, Genome, Singapore 138672
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
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

Nature, 2010, vol. 464, issue 7286, 292-296

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.

Date: 2010
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DOI: 10.1038/nature08792

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