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Etiology of congenital agammaglobulinemia: Growth defect of precursor B lymphoblastoid cell lines and immunoglobulin gene rearrangements.

Research Project

Project/Area Number 62480224
Research Category

Grant-in-Aid for General Scientific Research (B)

Allocation TypeSingle-year Grants
Research Field Pediatrics
Research InstitutionTOHOKU UNIVERSITY

Principal Investigator

TSUCHIYA Shigeru  Research Institute for Tuberculosis and Cancer, Tohoku University, 抗酸菌病研究所, 助教授 (30124605)

Co-Investigator(Kenkyū-buntansha) 寺沢 政彦  東北大学, 抗酸菌病研究所, 助手 (80192201)
SATO Tetsuo  Research Institute for Tuberculosis and Cancer, Tohoku University, 抗酸菌病研究所, 助手 (90170761)
KONNO Tasuke  Research Institute for Tuberculosis and Cancer, Tohoku University, 抗酸菌病研究所, 教授 (00004846)
TERASAWA Masahiko  Research Institute for Tuberculosis and Cancer, Tohoku University
Project Period (FY) 1987 – 1988
Project Status Completed (Fiscal Year 1988)
Budget Amount *help
¥6,200,000 (Direct Cost: ¥6,200,000)
Fiscal Year 1988: ¥2,000,000 (Direct Cost: ¥2,000,000)
Fiscal Year 1987: ¥4,200,000 (Direct Cost: ¥4,200,000)
Keywordscongenital agammaglobulinemia / Precursor B cell line / IgD / モノクローナル抗体 / IgD / 細胞クローニング / K+λ産生細胞クローン / K+入鎖産生細胞クローン / EBウイルス / プレBリンパ芽球様細胞株 / 表面マーカー
Research Abstract

Epstein-Barr virus (EBV) induced B lymphoblastoid cell lines (B-LCL) were established from bone marrow cells of the patients with congenital agammaglobulinemia (CAG).We found several immature B-LCL, some of which possess no cell-surface and cytoplastic immunoglobulins, or possess only cytoplasmic heavy chains. We examined the mode of expression of B-cell associated antigens on EBV-induced B-LCL and failed to detect any CAG specific and developmental stage-specific expression of known B-cell related antigens on those B-LCL. These facts indicate that expression of B cell associated antigens on EBV-induced B-LCL does not depend on the developmental stage of B cells, rather depend on the state of EBV infections.
If we can detect LCL which express immunoglobulins with very unusual manner, those LCL will probably give us oppotunities to elucidate the etiology of CAG. Fortunately we found 2 such cell lines, K5 and K4, from the same CAG patient. K5 was very unique because they expressed only IgD (delea,lambda) on the surface and produced IgD. K4 was also very unique because they produced lambda heavy chain, and both kappa and lambda light chains. Cell cloning experiments repeated 3 times definitely confirmed that K4 cells produced both kappa and lambda light chains with single mu chains. The pattern of immunoglobolin production seen on K4 and K5 was probably the first ones in the literature. We are going to clone immunoglobuline genes of K4 and K5, and to elucidate the genetic mechanism of the expression of unusual immunoglobulins.

Report

(3 results)
  • 1988 Annual Research Report   Final Research Report Summary
  • 1987 Annual Research Report
  • Research Products

    (16 results)

All Other

All Publications (16 results)

  • [Publications] Minegishi,M.,et al.: Tohoku J.exp.Med.151. 283-292 (1987)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Kojima,H.,et al.: BBRC. 143. 716-722 (1987)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Minegish,N.,et al.: Tohoku J.exp.Med.154. 143-148 (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Minegishi,M.,et al.: Leukemia Research. 12. 227-232 (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Minegishi,M.,et al.: Leukemia Research. 12. (1988)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] 土屋滋: Medical Immunology. (1989)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Minegishi, M. et al.: "Establishment of five human malignant non-T lymphoid cell lines and mixed lymphocyte-tumor reaction." Tohoku J. exp. Med.151. 283-292 (1987)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Kojima, H. et al.: "Predefined gene transfer for expression of a glycosphingolipid antigen by transfection with a cosmid genomic library prepared from a cell line in which the specific glyco-sphingolipid is highly expressed." BBRC. 143. 716-722 (1987)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Minegishi, N. et al.: "Chronic granulomatous disease with neutrophil membrane cytochrome b deficiency: Demonstration by immunochemical staining with monoclonal antibody." Tohoku J. exp. Med.154. 143-148 (1988)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Minegishi, M. et al.: "Characterization of a precursor T-cell line (THP) with rearranged T-cell precursor chain gene." Leukemia Research. 12. 227-232 (1988)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Minegishi, M. et al.: "Monoclonal antibody directed to human T-cell malignancy antigen." Leukemia Research. 12. (1988)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1988 Final Research Report Summary
  • [Publications] Minegishi,N;et al: Tohoku J.exp.Med.154. 143-148 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] Minegishi,M.;et al: Leukemia Research. 12. 227-232 (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] Minegishi,M.;et al: Leukemia Research. 12. (1988)

    • Related Report
      1988 Annual Research Report
  • [Publications] 土屋滋: 小児医学.

    • Related Report
      1988 Annual Research Report
  • [Publications] 土屋滋: Medical Immunology.

    • Related Report
      1988 Annual Research Report

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Published: 1987-04-01   Modified: 2016-04-21  

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