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FcgammaRIIB deficiency with Fas mutation is sufficient for the development of systemic autoimmune disease

Kaori Yajima1, Akira Nakamura, Akiko Sugahara

  • 1Department of Experimental Immunology, Institute of Development, Aging and Cancer, Tohoku University, Sendai, Japan.

European Journal of Immunology
|April 3, 2003
PubMed

Insights

The inhibitory Fc gamma RIIB gene acts as a critical suppressor of systemic lupus erythematosus (SLE). Its absence in mice with a Fas mutation leads to severe autoimmune disease, highlighting its role in SLE suppression.

Area of Science:

  • Immunology
  • Autoimmunity
  • Genetics

Background:

  • MRL.Fas(lpr/lpr) mice develop systemic autoimmune diseases like lupus nephritis due to a Fas mutation.
  • C57BL/6 (B6).Fas(lpr/lpr) mice do not develop these disorders, suggesting suppressor genes in B6 mice control disease onset.

Purpose of the Study:

  • To identify the suppressor gene(s) in B6 mice that control the onset and exacerbation of autoimmune diseases.
  • To investigate the role of the inhibitory Fc receptor for IgG (Fc gamma RIIB) as a critical suppressor of systemic lupus erythematosus (SLE).

Main Methods:

  • Generation of Fc gamma RIIB-deficient B6.Fas(lpr/lpr) (B6.IIB(-/-)Fas(lpr/lpr)) mice.
  • Analysis of autoimmune disease development, autoantibody production, and survival in these mice.

Main Results:

  • Fc gamma RIIB-deficient B6.Fas(lpr/lpr) mice developed systemic autoimmune diseases, including anti-DNA and anti-type II collagen autoantibodies, cryoglobulinemia, immune complex glomerulonephritis, and arthritis.
  • These mice exhibited enhanced autoantibody production by B cells, leading to fatal lupus nephritis and a shortened lifespan.
  • Fc gamma RIIB deletion in conjunction with a Fas mutation was sufficient to induce systemic autoimmunity in B6 mice.

Conclusions:

  • The gene for Fc gamma RIIB is a critical suppressor of systemic lupus erythematosus (SLE).
  • The inhibitory signaling cascade mediated by Fc gamma RIIB plays a crucial role in suppressing SLE.
  • Fc gamma RIIB deficiency combined with a Fas mutation is sufficient to cause systemic autoimmunity in the B6 mouse model.

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