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DEVELOPMENTAL STUDY OF ENTERIC NERVOUS SYSTEM BY NEURAL CREST CELLS FOR CONGENITAL AGANGLIONOSIS

Research Project

Project/Area Number 13671870
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Pediatric surgery
Research InstitutionKyoto Prefectural University of Medicine

Principal Investigator

SHIMOTAKE Takashi  KYOTO PREFECTURAL UNIVERSITY OF MEDICINE, CHILDREN RESEARCH HOSPITAL, DIVISION OF SURGERY, ASSISTANT, 医学研究科, 助手 (90254341)

Project Period (FY) 2001 – 2003
Project Status Completed (Fiscal Year 2003)
Budget Amount *help
¥4,100,000 (Direct Cost: ¥4,100,000)
Fiscal Year 2003: ¥1,000,000 (Direct Cost: ¥1,000,000)
Fiscal Year 2002: ¥1,000,000 (Direct Cost: ¥1,000,000)
Fiscal Year 2001: ¥2,100,000 (Direct Cost: ¥2,100,000)
KeywordsHIRSCHSPRUNG DISEASE / NEURAL CREST CELL / ENTERIC NERVOUS SYSTEM / PELVIC FLOOR MUSCLE / 神経堤細胞 / ヒルシュスプリング病 / 発生学 / 分化誘導
Research Abstract

The pathogenesis of Hirschsprung's disease was studied with respect to possible developmental induction of neural crest-derived cells by neural growth factors. In this preliminary study using an experimental culture system, neural crest-derived cells were developed in the presence of neural growth factors. In children with total colonic aganlionosis with small bowel involvement or near total intestinal aganglionosis, the affected aganglionic bowel segment is treated or modified by primary pull-through, proximal small bowel myectomy-myotomy, or possible bowel elongation technique. Pediatric patients with total intestinal aganglionosis are often fatal in spite of varied aggressive therapeutic challenge including total parenteral nutrition, intestinal transplantation, and neural crest maturation therapy. In the future treatment, affected aganglionic or transitional hypoganglionic bowel may be treated by appropriate neural growth stimulating factors to promote the maturation of enteric nervous system

Report

(4 results)
  • 2003 Annual Research Report   Final Research Report Summary
  • 2002 Annual Research Report
  • 2001 Annual Research Report
  • Research Products

    (30 results)

All Other

All Publications (30 results)

  • [Publications] Shimotake T, Tomiyama H, Aoi S, Iwai N: "Discrepancy between macroscopic and microscopic transitional zones in Hirschsprung's disease with reference to the type of RET/GDNF/NTN gene mutation."Journal of Pediatric Surgery. 38(6). 698-701 (2003)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Shimotake T, Go S, Tomiyama H, Aoi S, Iwai N.: "Proximal jejunostomy with or without myectomy-myotomy modification in five infants with total intestinal aganglionosis: An experience with surgical treatments in a single institution."Journal of Pediatric Surgery. 37(6). 835-839 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Tomiyama H, Shimotake T, Ono S, Kimura 0, Tokiwa K, Iwai N.: "Relationship between the type of RET/GDNF/NTN or SOX10 gene mutations and long-term results after surgery for total colonic aganglionosis with small bowel involvement."Journal of Pediatric Surgery. 36(11). 1685-1688 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Pingault V, Girard M, Bondurand N, Dorkins H, Shimotake T, Verma I, Baumann C, Goossens M: "SOX10 mutations in chronic intestinal pseudo-obstruction suggest a complex physiopathological mechanism"Human Genetics. 111(2). 198-206 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Bitoh Y, Shimotake T, Sasaki Y, Iwai N.: "Development of the pelvic floor muscles of nurine embryos with anorectal malformations."Journal of Pediatric Surgery. 37(2). 224-227 (2002)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Shimotake T, Tomiyama H, Aoi S, Iwai N: "Discrepancy between macroscopic and microscopic transitional zones in Hirschsprung's disease with reference to the type of RET/GDNF/NTN gene mutation."Journal of Pediatric Surgery. 38. 698-701 (2003)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Shimotake T, Go S, Tomiyama H, Aoi S, Iwai N: "Proximal jejunostomy with or without myectomy-myotomy modification in five infants with total intestinal aganglionosis : An experience with surgical treatments in a single institution."Journal of Pediatric Surgery. 37. 835-839 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Tomiyama H, Shimotake T, Ono S, Kimura O, Tokiwa K, Iwai N.: "Relationship between the type of RET/GDNF/NTN or SOX10 gene mutations and long-term results after surgery for total colonic aganglionosis with small bowel involvement"Journal of Pediatric Surgery. 36. 1685-1688 (2001)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Pingault V, Girard M, Bondurand N, Dorkins H, Shimotake T, Verma I, Baumann C, Goossens M: "SOX10 mutations in chronic intestinal pseudo-obstruction suggest a complex physiopathological mechanism"Human Genetics. 111. 198-206 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Bitoh Y, Shimotake T, Sasaki Y, Iwai N: "Development of the pelvic floor muscles of murine embryos with anorectal malformations."Journal of Pediatric Surgery. 37. 224-227 (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Bitoh Y, Shimotake T, Kubota Y, Kimura O, Iwai N: "Impaired distribution of retinoic acid receptors in the hindgut-tailgut region of murine embryos with anorectal malformations."Journal of Pediatric Surgery. 36. 377-380 (2001)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Shimotake T, Aoi S, Tomiyama H, Iwai N: "DPC-4 (Smad-4) and K-ras gene mutations in biliary tract epithelium in children with anomalous pancreaticobiliary ductal union."Journal of Pediatric Surgery. 38. 694-697 (2003)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2003 Final Research Report Summary
  • [Publications] Shimotake T, Tomiyama H, Aoi S, Iwai N: "Discrepancy between macroscopic and microscopic transitional zones in Hirschsprung's disease with reference to the type of RET/GDNF/NTN gene mutation."Journal of Pediatric Surgery. 38(6). 698-701 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] Shimotake T, Go S, Tomiyama H, Aoi S, Iwai N.: "Proximal jejunostomy with or without myectomy-myotomy modification in five infants with total intestinal aganglionosis : An experience with surgical treatments in a single institution."Journal of Pediatric Surgery. 37(6). 835-839 (2002)

    • Related Report
      2003 Annual Research Report
  • [Publications] Tomiyama H, Shimotake T, Ono S, Kimura O, Tokiwa K, Iwai N.: "Relationship between the type of RET/GDNF/NTN or SOX10 gene mutations and long-term results after surgery for total colonic aganglionosis with small bowel involvement."Journal of Pediatric Surgery. 36(11). 1685-1688 (2001)

    • Related Report
      2003 Annual Research Report
  • [Publications] Pingault V, Girard M, Bondurand N, Dorkins H, Shimotake T, Verma I, Baumann C, Goossens M: "SOX10 mutations in chronic intestinal pseudo-obstruction suggest a complex physiopathological mechanism"Human Genetics. 111(2). 198-206 (2002)

    • Related Report
      2003 Annual Research Report
  • [Publications] Shimotake T, Aoi S, Tomiyama H, Iwai N: "DPC-4(Smad-4)and K-ras gene mutations in biliary tract epithelium in children with anomalous pancreaticobiliary ductal union."Journal of Pediatric Surgery. 38(6). 694-697 (2003)

    • Related Report
      2003 Annual Research Report
  • [Publications] Bitoh Y, Shimotake T, Sasaki Y, Iwai N.: "Development of the pelvic floor muscles of murine embryos with anorectal malformations."Journal of Pediatric Surgery. 37(2). 224-227 (2002)

    • Related Report
      2003 Annual Research Report
  • [Publications] Tomiyama H, Shimotake T, Ono S, Kimura O, Tokiwa K, Iwai N.: "Relationship between the type of RET/GDNF/NTN or SOX10 gene mutations and long-term results after surgery for total colonic aganglionosis with small bowel involvement"Journal of Pediatric Surgery. 36(11). 1685-1688 (2001)

    • Related Report
      2002 Annual Research Report
  • [Publications] Shimotake T, Go S, Tomiyama H, Aoi S, Iwai N.: "Proximal jejunostomy with or without myectomy-myotomy modification in five infants with total intestinal aganglionosis : An experience with surgical treatments in a single institution"Journal of Pediatric Surgery. 37(6). 835-839 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] Shimotake T, Go S, Inoue K, Tomiyama H, Iwai N.: "A homozygous missense mutation in the tyrosine E Kinase domain of the RET proto-oncogene in an infant with total intestinal aganglionosis"American Journal of Gastroenterology. 96(4). 1286-1291 (2001)

    • Related Report
      2002 Annual Research Report
  • [Publications] Pingault V, Girard M, Bondurand N, Dorkins H, Shimotake T, Verma I, Baumann C, Goossens M: "SOX10 mutations in chronic intestinal pseudo-obstruction suggest a complex physiopathological mechanism"Human Genetics. 111(2). 198-206 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] Inoue K, Shimotake T, Tomiyama H, Iwai N.: "Mutational analysis of the RET and GDNF gene in children with hypoganglionosis"European Journal of Pediatric Surgery. 11(2). 120-123 (2001)

    • Related Report
      2002 Annual Research Report
  • [Publications] Bitoh Y, Shimotake T, Saski Y, Iwai N.: "Development of the pelvic floor muscles of murine embryos with anorectal malformations"Journal of Pediatric Surgery. 37(2). 224-227 (2002)

    • Related Report
      2002 Annual Research Report
  • [Publications] Bitoh Y, Shimotake T, Sasaki Y, et al.: "Development of the pelvic floor muscles of murine embryos with anorectal malformations"Journal of Pediatric Surgery. 37(2). 224-227 (2002)

    • Related Report
      2001 Annual Research Report
  • [Publications] Bitoh Y, Shimotake T, Kubota Y, et al.: "Impaired distribution of retinoic acid receptors in the hindgut-tailgut region of murine embryos with anorectal malformations"Journal of Pediatric Surgery. 36(2). 377-380 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] Tomiyama H, Shimotake T, Ono S, et al.: "Relationship between the type of RET/GDNF/NTN or SOX10 gene mutations and long-term results after surgery fortotal colonic aganglionosis with small bowel involvement"Journal of Pediatric Surgery. 36(11). 1685-1688 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] Shimotake T, Go S, Tomiyama H, et al.: "Proximal jejunostomy with or without myectomy-myotomy modification in 5 infants with total intestinal aganglionosis-an experience of surgical treatments in a single institution-"Journal of Pediatric Surgery. (in press). (2002)

    • Related Report
      2001 Annual Research Report
  • [Publications] Shimotake T., Go S., Inoue K., et al.: "A homozygous missense mutation in the tyrosine kinase domain of the RET proto-oncogene in an infant with total intestinal aganglionosis"American Journal of Gastroenterology. 96(4). 1286-1291 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] Inoue K, Shimotake T, Tomiyama H, et al.: "Mutational analysis of the RET and GDNF genes in children with hypoganglionosis"European Journal of Pediatric Surgery. 11(2). 120-123 (2001)

    • Related Report
      2001 Annual Research Report

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Published: 2001-04-01   Modified: 2016-04-21  

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