Gene Analysis and Neural Crest Stem Cell Transplant Therapy for Hirschsprung's disease and the Related disorders.

• Project/Area Number: 24791890
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Pediatric surgery
• Research Institution: Keio University
• Principal Investigator: FUJIMURA Takumi 慶應義塾大学, 医学部, 助教 (80573443)
• Project Period (FY): 2012-04-01 – 2015-03-31
• Project Status: Completed (Fiscal Year 2014)
• Budget Amount: ¥4,290,000 (Direct Cost: ¥3,300,000、Indirect Cost: ¥990,000); Fiscal Year 2014: ¥650,000 (Direct Cost: ¥500,000、Indirect Cost: ¥150,000); Fiscal Year 2013: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000); Fiscal Year 2012: ¥2,600,000 (Direct Cost: ¥2,000,000、Indirect Cost: ¥600,000)
• Keywords: 再生医療 / 神経堤細胞 / 小児難治性疾患 / 腸管運動不全 / 細胞移植治療 / ヒルシュスプルング病 / 幹細胞 / 幹細胞移植

Outline of Final Research Achievements

A lack of enteric ganglion cells is the main feature of Hirschsprung's disease (HSCR). Some patients were not fully recovered because of the long aganglionic segment. For the future transplantation therapy, the neural crest derived cell (NCC) is one of the bestcandidates which can give rise to the neural lineage cells.The transplanted cells were detected in the aganglonic segmenst of the gut for several months after operation and they showed the neural lineage differentiation. Only HSCR model mice from transplanted group showed an improvement of the bowel function.Transplanted NCCs survived and facilitated the improvement of the gut motility in the HSCR model. These results suggest that usefulness of the cell transplantation therapy for future HSCR treatment.

Research Products

• [Presentation] Transplanted Neural Crest Cells Support the Improvement of the Motility for a Mouse Model of Hirschsprung's Disease (2015)
  • Author(s): Takumi Fujimura
  • Organizer: ENS meeting 2015
  • Place of Presentation: Mainport Hotel(Rotterdam, Netherlands)
  • Year and Date: 2015-04-19 – 2015-04-22
• [Presentation] 腸管運動不全モデルマウスを用いた神経堤幹細胞移植治療の有用性に関する研究 (2015)
  • Author(s): 藤村 匠
  • Organizer: 第115回日本外科学会学術集会
  • Place of Presentation: 名古屋国際会議場（愛知県名古屋市）
  • Year and Date: 2015-04-16 – 2015-04-18
• [Presentation] 腸管運動不全に対する神経堤幹細胞を用いた細胞移植に関する研究 (2014)
  • Author(s): 藤村 匠
  • Organizer: 第50回日本移植学会
  • Place of Presentation: 京王プラザホテル（東京都新宿区）
  • Year and Date: 2014-09-10 – 2014-09-12
• [Presentation] 神経堤由来細胞を蛍光標識したマウスを用いた薬剤性ヒルシュスプルング病モデルの有用性に関する検討 (2013)
  • Author(s): 藤村 匠
  • Organizer: 第50回日本小児外科学会総会
  • Place of Presentation: 東京
• [Presentation] Evaluation of the drug-induced Hirschsprung's disease model with the genetic labeling for the neural crest derivatives (2013)
  • Author(s): Takumi Fujimura
  • Organizer: Neuro2013
  • Place of Presentation: 京都
• [Presentation] 神経堤由来細胞を蛍光標識したマウスを用いた薬剤性ヒルシュスプルング病モデルの有用性に関する検討 (2013)
  • Author(s): 藤村 匠
  • Organizer: 第50回小児外科学会総会
  • Place of Presentation: 京王プラザホテル 本館５Ｆ
• [Presentation] Evaluation of the drug-induced Hirschsprung's disease model with the genetic labeling for the neural crest derivatives (2013)
  • Author(s): Takumi Fujimura
  • Organizer: Neuro2013
  • Place of Presentation: 国立京都国際会館
• [Presentation] 神経堤由来細胞を蛍光標識したマウスを用いた薬剤性ヒルシュスプルング病モデルの有用性に関する検討
  • Author(s): 藤村 匠
  • Organizer: 第19回大腸肛門機能障害研究会
  • Place of Presentation: 東京
• [Presentation] 神経堤由来細胞を蛍光標識したマウスを用いた薬剤性ヒルシュスプルング病モデルの有用性に関する検討
  • Author(s): 藤村 匠
  • Organizer: 第44回小児消化管研究会
  • Place of Presentation: 大阪
• [Presentation] 腸管不全に対する神経堤幹細胞を用いた細胞移植治療に関する研究
  • Author(s): 藤村 匠
  • Organizer: 第26回日本小腸移植研究会
  • Place of Presentation: 栃木
• [Book] 小児外科 (2013)
  • Author(s): 藤村 匠
  • Total Pages: 5
  • Publisher: 東京医学社