Abrogation of the alternative complement pathway by targeted deletion of murine factor B

  • Mitsuru Matsumoto
    Department of Internal Medicine, Department of Pediatrics, Center for Immunology, and Howard Hughes Medical Institute, Washington University School of Medicine, St. Louis, MO 63110; Department of Immunology, G. D. Searle and Company, St. Louis, MO 63198; and First Department of Internal Medicine, School of Medicine, Ehime University, Shigenobu, Ehime 791-02, Japan
  • Wataru Fukuda
    Department of Internal Medicine, Department of Pediatrics, Center for Immunology, and Howard Hughes Medical Institute, Washington University School of Medicine, St. Louis, MO 63110; Department of Immunology, G. D. Searle and Company, St. Louis, MO 63198; and First Department of Internal Medicine, School of Medicine, Ehime University, Shigenobu, Ehime 791-02, Japan
  • Antonella Circolo
    Department of Internal Medicine, Department of Pediatrics, Center for Immunology, and Howard Hughes Medical Institute, Washington University School of Medicine, St. Louis, MO 63110; Department of Immunology, G. D. Searle and Company, St. Louis, MO 63198; and First Department of Internal Medicine, School of Medicine, Ehime University, Shigenobu, Ehime 791-02, Japan
  • Joseph Goellner
    Department of Internal Medicine, Department of Pediatrics, Center for Immunology, and Howard Hughes Medical Institute, Washington University School of Medicine, St. Louis, MO 63110; Department of Immunology, G. D. Searle and Company, St. Louis, MO 63198; and First Department of Internal Medicine, School of Medicine, Ehime University, Shigenobu, Ehime 791-02, Japan
  • Jena Strauss-Schoenberger
    Department of Internal Medicine, Department of Pediatrics, Center for Immunology, and Howard Hughes Medical Institute, Washington University School of Medicine, St. Louis, MO 63110; Department of Immunology, G. D. Searle and Company, St. Louis, MO 63198; and First Department of Internal Medicine, School of Medicine, Ehime University, Shigenobu, Ehime 791-02, Japan
  • Xuefeng Wang
    Department of Internal Medicine, Department of Pediatrics, Center for Immunology, and Howard Hughes Medical Institute, Washington University School of Medicine, St. Louis, MO 63110; Department of Immunology, G. D. Searle and Company, St. Louis, MO 63198; and First Department of Internal Medicine, School of Medicine, Ehime University, Shigenobu, Ehime 791-02, Japan
  • Shigeru Fujita
    Department of Internal Medicine, Department of Pediatrics, Center for Immunology, and Howard Hughes Medical Institute, Washington University School of Medicine, St. Louis, MO 63110; Department of Immunology, G. D. Searle and Company, St. Louis, MO 63198; and First Department of Internal Medicine, School of Medicine, Ehime University, Shigenobu, Ehime 791-02, Japan
  • Tunde Hidvegi
    Department of Internal Medicine, Department of Pediatrics, Center for Immunology, and Howard Hughes Medical Institute, Washington University School of Medicine, St. Louis, MO 63110; Department of Immunology, G. D. Searle and Company, St. Louis, MO 63198; and First Department of Internal Medicine, School of Medicine, Ehime University, Shigenobu, Ehime 791-02, Japan
  • David D. Chaplin
    Department of Internal Medicine, Department of Pediatrics, Center for Immunology, and Howard Hughes Medical Institute, Washington University School of Medicine, St. Louis, MO 63110; Department of Immunology, G. D. Searle and Company, St. Louis, MO 63198; and First Department of Internal Medicine, School of Medicine, Ehime University, Shigenobu, Ehime 791-02, Japan
  • Harvey R. Colten
    Department of Internal Medicine, Department of Pediatrics, Center for Immunology, and Howard Hughes Medical Institute, Washington University School of Medicine, St. Louis, MO 63110; Department of Immunology, G. D. Searle and Company, St. Louis, MO 63198; and First Department of Internal Medicine, School of Medicine, Ehime University, Shigenobu, Ehime 791-02, Japan

書誌事項

公開日
1997-08-05
DOI
  • 10.1073/pnas.94.16.8720
公開者
Proceedings of the National Academy of Sciences

この論文をさがす

説明

<jats:p>To investigate the role of complement protein factor B (Bf) and alternative pathway activity<jats:italic>in vivo</jats:italic>, and to test the hypothesized potential genetic lethal effect of Bf deficiency, the murine Bf gene was interrupted by exchange of exon 3 through exon 7 (including the factor D cleaving site) with the<jats:italic>neo</jats:italic><jats:sup>r</jats:sup>gene. Mice heterozygous for the targeted Bf allele were interbred, yielding Bf-deficient offspring after the F<jats:sub>1</jats:sub>generation at a frequency suggesting that Bf deficiency<jats:italic>alone</jats:italic>has no major effect on fertility or fetal development. However, in the context of one or more genes derived from the 129 mouse strain, offspring homozygous for Bf deficiency were generated at less than expected numbers (<jats:italic>P</jats:italic>= 0.012). Bf-deficient mice showed no gross phenotypic difference from wild-type littermates. Sera from Bf-deficient mice lacked detectable alternative complement pathway activity; purified mouse Bf overcame the deficit. Classical pathway-dependent total hemolytic activity was lower in Bf-deficient than wild-type mice, possibly reflecting loss of the alternative pathway amplification loop. Lymphoid organ structure and IgG1 antibody response to a T-dependent antigen appeared normal in Bf-deficient mice. Sensitivity to lethal endotoxic shock was not significantly altered in Bf-deficient mice. Thus, deficiency of Bf and alternative complement activation pathway led to a less dramatic phenotype than expected. Nevertheless, these mice provide an excellent model for the assessment of the role of Bf and the alternative pathway in host defense and other functions<jats:italic>in vivo</jats:italic>.</jats:p>

収録刊行物

被引用文献 (4)*注記

もっと見る

詳細情報 詳細情報について

問題の指摘

ページトップへ