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Analysis of gene defects related to NK cell deficiency : Establishment of the disorder as a new immunodeficiency

Research Project

Project/Area Number 08457222
Research Category

Grant-in-Aid for Scientific Research (B)

Allocation TypeSingle-year Grants
Section一般
Research Field Pediatrics
Research InstitutionShinshu University

Principal Investigator

KOMIYAMA Atsushi  Shinshu Univ.School Med., Dept.Pediatr., Professor, 医学部, 教授 (50020798)

Co-Investigator(Kenkyū-buntansha) AGEMATSU Kazunaga  Shinshu Univ.Hosp., Dept.Pediatr., Associate Professor, 医学部附属病院, 講師 (60262721)
KOIKE Kenichi  Shinshu Univ.School Med., Dept.Pediatr., Assistant Professor, 医学部, 助教授 (40143979)
Project Period (FY) 1996 – 1997
Project Status Completed (Fiscal Year 1997)
Budget Amount *help
¥4,000,000 (Direct Cost: ¥4,000,000)
Fiscal Year 1997: ¥1,900,000 (Direct Cost: ¥1,900,000)
Fiscal Year 1996: ¥2,100,000 (Direct Cost: ¥2,100,000)
KeywordsNK cell / NK cell deficiency / familial NK cell deficiency / Chediak-Higashi syndrome / perforin / Fas / Fas ligand / immunodericiency
Research Abstract

The present studies were conducted to analyze the clinical chatacteristics and gene defects of natural killer (NK) cell deficiencies.
1. Familial NK cell deficieny (Komiyama, A., Pediatr., 85 : 323,1990)
(1) The hypersusceptibility to infections appears to be improved with age, with a gradual increase in the number of circulating rdT cells.
(2) The patients have CD56+ cells, all of which are CD3+CD56+ cells but not CD3-CD56+ cells. These results indicate that this disorder is due to the absence of circulating NK cells.
(3) Then, next studies were performed to examine the proliferative capacity of NK cell precursors by culturing the bone marrow cells in vitro. We failed to culture CD3-CD56+ cells in the presence of a combination of many cytokines or feeder layrs + IL-2 in the culture system. Further studies are necessary to elucidate the defects.
(4) The expression of perforin mRNA was normal in the cytotoxic T cells.
2. Chediak-Higashi syndrome
(1) NK cell activity was defective against K562 cells but almost normal against Jurkat cells. The latter activity was not exerted by tumor necrosis factor-a.
(2) The perfor in-mediated cytotoxicity was impaired, but its mRNA was normally expressedin the NK cells.
(3) The Fas mRNA expression was normal, and Fas/Fas ligand-mediated cytotoxicity via apoptosis was normal.
3.Other disorders such as hemophagocytic syndrome, systemic lupus erythematosus, and NK cell abnormality related to the Chernobyl accident.
The NK cell deficiencies in these disordeers were similarly analyzed in the present studies.

Report

(3 results)
  • 1997 Annual Research Report   Final Research Report Summary
  • 1996 Annual Research Report
  • Research Products

    (44 results)

All Other

All Publications (44 results)

  • [Publications] Sawai, N.Komiyama, A.: "Aberrant growth of GM-progenitors in juvenile CML in serum-free culture." Exp.Hematol.24. 116-122 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Yasui, K.Komiyama, A.: "Effects of high-dose G-CSF on neutrophil functions." Brit.J.Haematol.92. 571-573 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Yabuhara, A.Komiyama, A.: "A killing defect of NK cells as an underlying immunologic abnormality in childhood SLE." J.Rheumatol.23. 171-177 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Yasui, K.Komiyama, A.: "Successful treatment of Behcet disease with pentoxifylline." Ann.Intern.Med.124. 891-893 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Yang, F-C.Komiyama, A.: "CD27/CD70 interaction directly induces NK cell killing activity." Immunology. 88. 289-293 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Okumura, N.Komiyama, A.: "Chemotactic and chemokinetic activities of stem cell factor on murine hematopoietic progenitors." Blood. 87. 4100-4108 (1996)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Agematsu, K.Komiyama, A.: "B cell subpopulations separated by CD27 and crucial collaboration of CD27+ cells and helper T cells--." Eur.J.Immunol.27. 2073-2079 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Shiohara, M.Komiyama, A.: "P21^<WAF1> mutations and human malignancies." Leukemia Lymphoma. 26. 35-41 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Yasui, K.Komiyama, A.: "Theophylline accelerates human granulocyte apoptosis not via phosphodiestarase inhibition." J.Clin.Invest.100. 1677-1684 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Higuchi, T.Koike, K.: "Proliferative and differentiative potential of thrombopoitin-responsive precursors." Exp.Hematol.25. 463-470 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Nakazawa, T.Agematsu, K.: "Later development of fas ligand-mediated cytotoxicity as compared with granule-mediated---." Immunology. 92. 180-187 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Shiohara, M.Komiyama, A.: "Cytostatic effect of TNFa on cancer cells is independent of p21^<WAF1>." Oncogene. 15. 1605-1609 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Agematsu, K.Komiyama, A.: "Generation of plasma cells from peripheral blood memory B cells." Blood. 91. 173-180 (1998)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] 安井 耕三, 小宮山 淳: "Chediak-Higashi症候群の責任遺伝子" 医学のあゆみ. 180. 510-511 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] 小池 健一, 小宮山 淳: "チェルノブイリ原発事故後のベラル-シ共和国に対する医療協力" 日本医事新報. 3818. 53-55 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] 小宮山 淳: "原発性免疫不全症候群の分類" 小児内科. 29. 901-908 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] 薮原 明彦, 小宮山 淳: "IgGサブクラス欠乏症" 医学のあゆみ. 182. 798-802 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] 小宮山 淳, 上松 一永: "小児科学-続発性免疫不全症" 白木和夫,前川喜平(編).医学書院, 分担8 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Yabuhara, A., Komiyama, A., et al.: "A killing defect of natural killer cells as an underlying immunologic abnormality in childhood systemic lupus erythematosus." J.Rheumatol.23. 171-177 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Yang, F-C., Komiyama, A., et al.: "CD27/CD70 interaction directly induces natural killer cell killing activity." Immunology. 88. 289-293 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Yasui, K., Komiyama, A., et al.: "Effects of high-dose granulocyte colony-stimulating factor on neutrophil functions." Brit.J.Haematol.92. 571-573 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Okumura, N,Komiyama, A., et al.: "Chemotactic and chimokinetic activities of stem cell factor on murine hematopoietic progenitor cells." Blood. 87. 4100-4108 (1996)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Agematsu, K., Komiyama, A., et al.: "B cell subpopulation separated by CD27 and crucial collaboration of CD27+ B cells and helper T cells in immunoglobulin production." Eur.J.Immunol.27. 2073-2079 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Yasui, K.Komiyama, A., et al.: "Theophylline accelerates human granulocyte apoptosis not via phosphodiesterase inhibition." J.Clin.Invest.100. 1677-1684 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Nakazawa, T., Agematsu, K., et al.: "Later development of Fas ligand-mediated cytotoxicity as compared with granule-mediated cytotoxicity during the maturation of natural killer cells." Immunology. 92. 180-187 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Agematsu, K., Komiyama, A., et al.: "Generation of plasma cells from peripheral blood memory B cells : Synergistic effect of interleukin-10 and CD27/CD70 interaction." Blood. 91. 173-180 (1998)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Agematsu,K. Komiyama,A.: "B cell subpopulations separated by CD27 and crucial collaboretion of CD27+ cells and helper T cells--." Eur.J.Immunol.27. 2073-2079 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Shiohara,M. Komiyama,A.: "P21^<WAF1> mutations and human malignancies." Leukemia Lymphoma. 26. 35-41 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Yasui,K. Komiyama,A.: "Theophylline accelerates human granulocyte apoptosis not via phosphodiestarase inhibition." J.Clin.Invest.100. 1677-1684 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Higuchi,T. Koide,K.: "Proliferative and differentiative potential of thrombopoitin-responsive precursors." Exp.Hematol.25. 463-470 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Nakazawa,T. Agematsu,K.: "Later development of fas ligand-mediated cytotoxicity as compared with granule-mediated---." Immunology. 92. 180-187 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Shiohara,M. Komiyama,A.: "Cytostatic effect of TNFa on cancer cells is independent of p21^<WAF1>." Oncogene. 15. 1605-1609 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Agematsu,K. Komiyama,A.: "Generation of plasma cells from peripheral blood memory B cells." Blood. 91. 173-180 (1998)

    • Related Report
      1997 Annual Research Report
  • [Publications] 安井耕三, 小宮山淳: "Chediak-Higashi症候群の責任遺伝子" 医学のあゆみ. 180. 510-511 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] 小池健一, 小宮山淳: "チェルノブイリ原発事故後のベラル-シ共和国に対する医療協力" 日本医事新報. 3818. 53-55 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] 小宮山淳: "原発性免疫不全症候群の分類" 小児内科. 29. 901-908 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] 薮原明彦,小宮山淳: "IgGサブクラス欠乏症" 医学のあゆみ. 182. 798-802 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] 小宮山淳, 上松一永: "小児科学-続発性免疫不全症(分担)" 白木純夫 前川善平(編),医学書院, 8 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Yang,F-C.Komiyama,A.: "CD27/CD70 interaction directly induces natural killer cell killing activity." Immunology. 88. 289-293 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Yabuhara,A.Komiyama,A.: "A killing defect of NK cells as an underlying immunologic abnormality in childhood SLE." J.Rheumatol.23. 171-177 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Yasui,K.Komiyama,A.: "Successful treatment of Bechet disease with pentoxifylline." Ann.Intern.Med.124. 891-893 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Okumura,N.Komiyama,A.: "Chemotactic and chemokinetic activities of stem cell factor on murine hematopoietic progenitor cells." Blood. 87. 4100-4108 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] 小池健一,小宮山淳: "血球貧食の機序と免疫学的異常" 小児内科. 28. 1581-1585 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] 安井耕三,小宮山淳: "Chediak-Higashi症候群の責任遺伝子" 医学のあゆみ. 180. 510-511 (1997)

    • Related Report
      1996 Annual Research Report

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Published: 1996-04-01   Modified: 2016-04-21  

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