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RESEARCH FOR COMPLETE RECONSTITUTION OF IMMUNOLOGICAL FUNCTIONS BY LYNPHOID STEM CELL TRANSFUSION IN CONGENITAL IMMUNODEFICIENCY.

Research Project

Project/Area Number 07670842
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Pediatrics
Research InstitutionTOKYO MEDICAL AND DENTAL UNIVERSITY,SCHOOL OF MEDICINE

Principal Investigator

OKAWA Hiroji  TOKYO MEDICAL AND DENTAL UNIVERSITY,SCHOOL OF MEDICINE,ASSOCIATE PROFESSOR, 医学部, 助教授 (90160431)

Project Period (FY) 1995 – 1996
Project Status Completed (Fiscal Year 1996)
Budget Amount *help
¥2,300,000 (Direct Cost: ¥2,300,000)
Fiscal Year 1996: ¥900,000 (Direct Cost: ¥900,000)
Fiscal Year 1995: ¥1,400,000 (Direct Cost: ¥1,400,000)
KeywordsSTEM CELL TRANSFUSION / LYMPHOID STEM CELL / SEVERE COMBINED IMMUNODEFICIENCY / WISKOTT-ALDRICH SYNDROME / CD34 POSITIVE CELL / IL2-Rgamma CHAIN / WAS PROTEIN / 巨核球コロニー / リンパ球幹細胞移植 / 免疫不全症 / CD34陽性細胞 / IgH遺伝子 / TCR遺伝子 / WASP遺伝子
Research Abstract

The cause of congenital immunodeficiencies is defective stem cell masturation to lymphoid sytem. Therefor the stem cell transfusion is a cuarative therapy for these lethal diseases. The aims of this study is to analyze the abnormalities of lymphoid system of immunodeficiencies and the capacity of stem cell proliferation and differentiation.
To gain more insight into the human B (-) SCID defect and its effect on the AB repertoire, we examined the IgH chain genes of the peripheral blood leaky B cells. Ab repertoire of IgH chain gene in human B (-) SCID was limited because of restricted junctional and combinatorial diversity and few somatic mutation. T cell reconstitution by haploidentical BMT does not restore the diversification of the Ig heavy chain gene in these patients. For the study of stem cell differentiation and proliferation activities, cord blood stem cells show more excellent activities than the other one and is thought to be better for the cureable BMT for congenital immunodeficiency.

Report

(3 results)
  • 1996 Annual Research Report   Final Research Report Summary
  • 1995 Annual Research Report
  • Research Products

    (19 results)

All Other

All Publications (19 results)

  • [Publications] Yoshiyuki Minekishi,Naoto Ishii,Hirotoshi Maeda,Shuji Takaki,Masahiro Tsuchida,Hiroji Okawa,Kazuo Sugamura,Junnichi Yata:"Three novel mutations in the Interleukin-2 receptor γchain gene in four Japanese patients with X-Linked severe combined immuno-deficiency." Human Genetics. 96. 681-683 (1995)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Yoshiyuki Minegishi,Naoto Ishii,Masahiro Tsuchida,Hiroji Okawa,Kazuo Sugamura,Junichi Yata:"T cell reconstitution by hap1-identical BMT dose not restore the diver sification of the Ig heavy chain gene in patients with X-linked SCID." Bone Marrow Transplantation. 16. 801-806 (1995)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] 大川洋二: "造血幹細胞の分化・増殖と細胞膜蛋白" 病理と臨床. 13・11. 1499-1509 (1995)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] 大川 洋二: "骨髄移植後にEpstein-Barr virus に関連したLymphoproliferative disorderを発症したWiskott-Aldrich症候群の1例" 日本小児血液学会雑誌. 10・3. 210-215 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Yoshiyuki Minegishi,Kunihiko Akagi,Ken-ichi Nishikawa,Hiroji Okawa,Junichi Yata:"Analysis of the CDR3 region of the rearrange ment IgH chain gene in patients with severe combined immunodeficiency and severe lymphopenia." Journal of Immunology. 156. 4666-4672 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Kiyoshi Kawabata,Masayuki Nagasawa,Tomohiro Morio,Hiroji Okawa,Junichi Yata:"Decreased α/βheterodimer among CD8 molecules of peripheral blood T cells in Wiskott-Aldrich syndrome." Clinical Immunology and lmmunopathology. 81. 129-135 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Yoshiyuki Minegishi, Naoto Ishii, Hirotoshi Maeda, Masahiro Tsuchida, Hiroji Okawa, Junichi Yada: "Three novel mutation in the Interleukin-2 Receptor gamma chain gene in four Japanese patients with X-linked severe combined immuno deficiency." Human.Genet.96. 681-683 (1995)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Yoshiyuki Minegishi, Naoto Ishii, Masahiro Tsuchida, Hiroji Okawa, Kazuo Sugamura, Junichi Yada: "T cell reconstitution by haplo identical BMT dose not restore the diversification of the Ig heavy chain gene in patients with X-linked SCID" Bone Marrow.Trans.16. 801-806 (1995)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Yoshiyuki Minegishi, Kumihiko Akagi, Kenichi Nishikawa, Hiroji Okawa, Junichi Yata: "Analysis of the CDR3 region of the rearrangement IgH gene in patients with severe combined immunodeficiency and severe lymphopenia." J.Immunolo. 156. 4666-4672 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Kiyoshi Kawabata, Masayuki Nagasawa, Tomohiro Morio, Hiroji Okawa, Junichi Yata: "Decreased alpha/beta heterodimer among CD8 molecules of peripheral blood T cells in Wiskott-Aldrich Syndrome" Clini.Immunolo.and Immunopatho.81. 129-135 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] Kunie Yoshida, Yoshiyuki Minegishi, Hiroji Okawa Junichi Yata: "Epstein-Barr virus associated malignant lymphoma with macroamylasemia and monoclonal gammopathy in patients with Wiskott-Aldrich syndrome" Pediat.Hematolo.and Oncolo.14. 85-89 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1996 Final Research Report Summary
  • [Publications] 1)Yoshiyuki Minegishi,Naoto Ishii,Hirotoshi Maeda,Shuji Takagi,Masahiro Tsuchida,Hiroji Okawa,Kazuo Sugamura,Junichi Yata:"Three novel mutations in the Interleukin-2 receptor γchain gene in four Japanese patients with X-linked severe combined immuno-deficiency." Human Genetics. 96. 681-683 (1995)

    • Related Report
      1996 Annual Research Report
  • [Publications] Yoshiyuki Minegishi,Naoto Ishii,Masahiro Tsuchida,Hiroji Okawa,Kazuo Sugamura,JUnichi Yata:"T cell reconstitution by hapl-identical BMT dose not restore the diver sification of the Ig heavy chain gene in patients with X-linked SCID." Bone Marrow Transplantation. 16. 801-806 (1995)

    • Related Report
      1996 Annual Research Report
  • [Publications] 大川洋二: "造血幹細胞の分化・増殖と細胞膜蛋白" 病理と臨床. 13・11. 1499-1509 (1995)

    • Related Report
      1996 Annual Research Report
  • [Publications] 今井耕輔、山本貴子、伊東祐之、吉田邦枝、前田浩利、梶原道子、高木秀二、大川洋二、平井蕘二、矢田純一:"骨髄移植後にEpstein-Barr virusに関連したLymphoproliferative disorderを発症したWiskott-Aldrich症候 群の1例" 日本小児血液学会雑誌. 10・3. 210-215 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Yoshiyuki Minegishi,Kunihiko Akagi,Ken-ichi Nishikawa Hiroji Okawa,Junichi Yata:"Analysis of the CDR3 region of the rearrange ment IgH chain gene in patients with severe combined immunodeficiency and severe lymphopenia." Journal of Immunology. 156. 4666-4672 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Kiyoshi Kawabata,Masayuki Nagasawa,Tomohiro Morio,Hiroji Okawa,Junichi Yata:"Decreased α/βheterodimer among CD8 molecules of peripheral blood T cells in Wiskott-Aldrich syndrome." Clinical Immunology and Immunopathology. 81. 129-135 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] H.Okawa: "Tcell reconstitution by haploidentical BMT in patient with X-SCID" Bone Marrow Transplantation. (1995)

    • Related Report
      1995 Annual Research Report
  • [Publications] H.Okawa: "Three novel mutation in the IL-2Rσ chain gene in four Japanes patient with X-linkeda SCID" Hum,Geneti. 96. 681-683 (1995)

    • Related Report
      1995 Annual Research Report

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Published: 1995-04-01   Modified: 2016-04-21  

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