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Ptpn11 deletion in a novel progenitor causes metachondromatosis by inducing hedgehog signalling

Author

Listed:
  • Wentian Yang

    (Brown University Alpert Medical School and Rhode Island Hospital)

  • Jianguo Wang

    (Brown University Alpert Medical School and Rhode Island Hospital)

  • Douglas C. Moore

    (Brown University Alpert Medical School and Rhode Island Hospital)

  • Haipei Liang

    (Brown University Alpert Medical School and Rhode Island Hospital)

  • Mark Dooner

    (Rhode Island Hospital and Brown University Alpert Medical School)

  • Qian Wu

    (University of Connecticut Health Center)

  • Richard Terek

    (Brown University Alpert Medical School and Rhode Island Hospital)

  • Qian Chen

    (Brown University Alpert Medical School and Rhode Island Hospital)

  • Michael G. Ehrlich

    (Brown University Alpert Medical School and Rhode Island Hospital)

  • Peter J. Quesenberry

    (Rhode Island Hospital and Brown University Alpert Medical School)

  • Benjamin G. Neel

    (Princess Margaret Cancer Center, University Health Network, University of Toronto, Toronto, Ontario M5G 1L7, Canada)

Abstract

Deletion of Ptpn11 in a newly defined mesenchymal progenitor population in the perichondral groove of Ranvier leads to metachondromatosis by increasing Indian hedgehog expression and activating hedgehog signalling, a process that can be reversed with the use of hedgehog pathway inhibitors.

Suggested Citation

  • Wentian Yang & Jianguo Wang & Douglas C. Moore & Haipei Liang & Mark Dooner & Qian Wu & Richard Terek & Qian Chen & Michael G. Ehrlich & Peter J. Quesenberry & Benjamin G. Neel, 2013. "Ptpn11 deletion in a novel progenitor causes metachondromatosis by inducing hedgehog signalling," Nature, Nature, vol. 499(7459), pages 491-495, July.
    • Handle: RePEc:nat:nature:v:499:y:2013:i:7459:d:10.1038_nature12396
    DOI: 10.1038/nature12396
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    Cited by:

    1. Fenli Shao & Qianqian Liu & Yuyu Zhu & Zhidan Fan & Wenjun Chen & Shijia Liu & Xiaohui Li & Wenjie Guo & Gen-Sheng Feng & Haiguo Yu & Qiang Xu & Yang Sun, 2021. "Targeting chondrocytes for arresting bony fusion in ankylosing spondylitis," Nature Communications, Nature, vol. 12(1), pages 1-14, December.
    2. Yuki Matsushita & Angel Ka Yan Chu & Chiaki Tsutsumi-Arai & Shion Orikasa & Mizuki Nagata & Sunny Y. Wong & Joshua D. Welch & Wanida Ono & Noriaki Ono, 2022. "The fate of early perichondrial cells in developing bones," Nature Communications, Nature, vol. 13(1), pages 1-17, December.
    3. Masayuki Tsukasaki & Noriko Komatsu & Takako Negishi-Koga & Nam Cong-Nhat Huynh & Ryunosuke Muro & Yutaro Ando & Yuka Seki & Asuka Terashima & Warunee Pluemsakunthai & Takeshi Nitta & Takashi Nakamura, 2022. "Periosteal stem cells control growth plate stem cells during postnatal skeletal growth," Nature Communications, Nature, vol. 13(1), pages 1-11, December.

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