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Genetic mechanisms of the complement deficiency associated with immune abnormalities

Research Project

Project/Area Number 02454186
Research Category

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

Allocation TypeSingle-year Grants
Research Field Immunology
Research InstitutionKanazawa University

Principal Investigator

TAKAHASHI Morinobu  Kanazawa University, Cancer Research Institute, Professor, がん研究所, 教授 (80019877)

Project Period (FY) 1990 – 1991
Project Status Completed (Fiscal Year 1991)
Budget Amount *help
¥6,900,000 (Direct Cost: ¥6,900,000)
Fiscal Year 1991: ¥3,100,000 (Direct Cost: ¥3,100,000)
Fiscal Year 1990: ¥3,800,000 (Direct Cost: ¥3,800,000)
KeywordsComplement deficiency / Molecular cloning / Mouse C4 / B2 suquence / RNA splicing / retrotransposon / レトロトランスポゾン / 補体第3成分(C3) / C3欠損症 / HRFー20 / 発作性夜間血色素尿症 / マウスC4 / RNAプロセシング / 転写調節
Research Abstract

1) In order to gain insight info the genetic mechanism of human C3 deficiency, mRNA and DNA extracted from peripheral blood were analyzed by Northern and Southern blotting. Furthermore, C3 gene of 16kb long was isolated from the patient DNA and subjected to extensive structural and functional analyzes. The combined results showed that the C3 deficient state of this patient is caused by genetic irregularities at the posttranscriptional and pretranslational level.
2) Mouse strains of H-2_k haplotype are invariably low C4 producers. Analysis of C4 mRNA from these mouse strains'revealed the presence of abnormal 200bp insert consisting of B2 repetitive sequence and part of intronic sequence. Subsequent structural. analyses of C4 genes isolated from low C4 producers showed the retrotransposon-like insertion of B2 sequent in the 13th intron of the gene, which probably accounts for the aberrant RNA splicing of the C4 transcript.

Report

(3 results)
  • 1991 Annual Research Report   Final Research Report Summary
  • 1990 Annual Research Report
  • Research Products

    (22 results)

All Other

All Publications (22 results)

  • [Publications] 中山 耕造他: "Posttrans criptional regulation of complement C4 gene in the low C4 producing strain of mouse" Immunogenetics. 31. 361-367 (1990)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1991 Final Research Report Summary
  • [Publications] Saーnga Pattanaki tsakul 他: "Three extra copies of C4ーRelated genes in Hー2^<W7> mice are C4ーSlp hybrict genes generated by multiple recombinational events" Immunogenetics. 32. 431-439 (1990)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1991 Final Research Report Summary
  • [Publications] 黄 志明他: "Differential expression of the five C4ーrelated genes of Hー2^<W7> mice" Immunogenetics. 33. 361-366 (1991)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1991 Final Research Report Summary
  • [Publications] 黄 志明他: "Evidence for the common evolutionary origin of the C4/Slp locus in the two wild mouseーderived haplotypes,W7 and W19" Immunogenetics. 35. (1992)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1991 Final Research Report Summary
  • [Publications] Saーnga Pattanaki tsakull他: "Aberrant splicing caused by the insertion of the B2 sequence into an intron of the complement C4 gene is the basis for low C4 production in Hー2^k mouse" Journal of Biological Chemistry. 267. (1992)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1991 Final Research Report Summary
  • [Publications] 野中 勝他: "The complete cDNA sequence of the third componont of complement(C3)of lamprey:implication of thiolester containing proteins" Journal of Immunology. 148. (1992)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1991 Final Research Report Summary
  • [Publications] K. Nakayama et al.: "Posttranscriptional regulation of the complement C4 gene in the low C4-producing strain of mouse." Immunogenetics. 31. 361-367 (1990)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1991 Final Research Report Summary
  • [Publications] S. Pattanakitsakul et al.,: "Three extra copies of C4-related genes in H-2^<w7> mice are C4/Slp hybrid genes generated by multiple recombinational events." Immunogenetics. 32. 431-439 (1990)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1991 Final Research Report Summary
  • [Publications] Z. M. Huang et al.: "Differential expression of the five C4-related genes of H-2^<w7> mice." Immunogenetics. 33. 361-366 (1991)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1991 Final Research Report Summary
  • [Publications] Z. M. Huang et al .: "Evidence for the common evolutionary origin of the C4/Slp Locus in the two wild mouse-derived haplotypes, W7 and W19." Immunogenetics. 35. (1992)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1991 Final Research Report Summary
  • [Publications] S. Pattanakitsakul et. al.: "Aberrant splicing caused by the insertion of the B2 sequence into an intron of the complement C4 gene is the basis for low C4 production in H-2^k mouse." Journal of Biological Chemistry. 267. (1992)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1991 Final Research Report Summary
  • [Publications] M. Nonaka et al.: "The complete cDNA sequence of the third complement (c3) of lamprey : implication of thiol ester containing Proteins." Journal of Immunology. 148. (1992)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1991 Final Research Report Summary
  • [Publications] 中山 耕造 他: "Postーtranscriptional regulation of the complement C4 gene in the low C4ーproducing strain of mouse" Immunogenetics. 31. 361-367 (1990)

    • Related Report
      1991 Annual Research Report
  • [Publications] Saーnga Pattanakitsakul 他: "Three extra copies of C4ーrelatecl genes in Hー2^<w7> mice are C41Slp hybrid genes generated by multiple recombinational euents" Immunogenetics. 32. 431-439 (1990)

    • Related Report
      1991 Annual Research Report
  • [Publications] 黄 志明 他: "Differential expression of the sive C4ーrelated genes of Hー2^<w7> mice" Immunogenetics. 33. 361-366 (1991)

    • Related Report
      1991 Annual Research Report
  • [Publications] 黄 志明 他: "Evidenee for the common evolutionary origin of the C41Slp locus in the two wild mousederived haplotypes,W7 and W19." Immunogenetics. 35. (1992)

    • Related Report
      1991 Annual Research Report
  • [Publications] Saーnga Pattanakitsakul 他: "Aberrant splicing caused by the insertion of the B2 sequence into an intron of the complement C4 gene is the basis for low C4 production in Hー2^k mouse" Journal of Biological Chemistry. 267. (1992)

    • Related Report
      1991 Annual Research Report
  • [Publications] 野中 勝 他: "The complete cDNA sequence of the third component of complement(C3)of lamprey:implication of thiolester containing proteins" Journal of Immunology. 148. (1992)

    • Related Report
      1991 Annual Research Report
  • [Publications] Kohzo Nakayama: "Postーtranscriptional regulation of complement C4 in low C4ーproducing strain of mouse" Immunogenetics. 31. 361-367 (1990)

    • Related Report
      1990 Annual Research Report
  • [Publications] Saーnga Pattanakitsakul: "Three extra copies of a C4ーrelated gene in Hー2^<w7> mice are C4/SCp hybrid genes generated by multiple recombinational events" Immunogenetics. 32. 431-439 (1990)

    • Related Report
      1990 Annual Research Report
  • [Publications] ZhiーMing Huang: "Differential expression of the five C4ーrelated genes of Hー2^<w7>" Immunogenetics.

    • Related Report
      1990 Annual Research Report
  • [Publications] JiaーHua Zheng: "Unusual processing of the preーmRNA for the fourth component of complement in low C4 producing strain of mouse"

    • Related Report
      1990 Annual Research Report

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Published: 1990-04-01   Modified: 2016-04-21  

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