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2015 年度 実績報告書

Patient-specific iPS cell-derived vessel smooth muscle cells of DD-EDS syndrome

研究課題

研究課題/領域番号 25860853
研究機関信州大学

研究代表者

岳 鳳鳴  信州大学, 学術研究院医学系, 助教 (20532865)

研究期間 (年度) 2013-04-01 – 2016-03-31
キーワードDD-EDS iPS cells / vessel smooth muscle / contrality function / vessel formation
研究実績の概要

During the period of 2015, more two specific DD-EDS iPS cells were established from the other two patients. Vessel smooth muscle cells were induced from iPS cells. And also, we examined the expression of contractile gene and protein, contractile function, and vessel formation.
We found that almost all the cells were positive for double staining with αSMA and calponin. The gene expressions of contractile smooth muscle marker (SMTN and TAGLN) were all lower in VSMCs derived from DD-EDS patient, compared with normal iPS-derived VSMCs. Reduced contractile response to vasoactive agonists such as endothelin-1 and carbachol were found in DD-EDS patient iPS-derived VSMCs compared with normal iPS-derived VSMCs.
Therefore, we can conclude that the insufficient contractile properties of VSMCs and abnormal blood vessel formation might be some reasons of subcutaneous hematoma which repeatedly happened in DD-EDS patient.

  • 研究成果

    (1件)

すべて 2016

すべて 学会発表 (1件) (うち国際学会 1件、 招待講演 1件)

  • [学会発表] Disease Model with iPS Cells from D4ST1-Deficient Ehlers-Danlos Syndrome (DD-EDS)2016

    • 著者名/発表者名
      Fengming Yue, Tomoki Kosho, Katsunori Sasaki
    • 学会等名
      International Symposium of Regenerative Medicine in Matsumoto
    • 発表場所
      Shinshu University Hospital, Matsumoto, Nagano
    • 年月日
      2016-04-02
    • 国際学会 / 招待講演

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公開日: 2017-01-06  

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