Development of ALS-like disease in SOD-1 mice deficient of B lymphocytes

J Neurol. 2009 Aug;256(8):1228-35. doi: 10.1007/s00415-009-5097-3. Epub 2009 Mar 12.


Several recent studies proposed a role for innate immunity and inflammation in the pathogenesis of amyotrophic lateral sclerosis (ALS). However, possible links, if any, between disease and adaptive immunity are poorly understood. The present study probed for the role of B cells in ALS disease using the G93A-SOD-1 transgenic mouse model. In agreement with other studies, we show here that autoantibodies are detectable in SOD-1 mice. However, SOD-1 B cells did not express any altered phenotype and exhibited indistinguishable responsiveness to immunogenic stimuli relative to wild-type B cells. This was obtained for B cells isolated before, during and after the onset of ALS-like disease. Finally, to obtain an in vivo conclusion, we generated SOD-1 mice that are deficient of B cells, by crossing SOD-1 mice with Igmu-deficient mice (muMT), where B cell development is blocked at the proB stage. The meteoric assays performed on a rota-rod clearly showed the development of ALS-like disease in SOD-1 mice that are deficient of B cells not differently than in control SOD-1 mice. Our results propose that B lymphocytes do not have a major role in the pathogenesis of ALS-like disease in SOD-1 mice.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyotrophic Lateral Sclerosis / genetics
  • Amyotrophic Lateral Sclerosis / immunology*
  • Amyotrophic Lateral Sclerosis / physiopathology
  • Animals
  • Antibody Formation / immunology*
  • Autoantibodies / immunology
  • Autoimmunity / genetics
  • Autoimmunity / immunology
  • B-Lymphocytes / immunology*
  • B-Lymphocytes / metabolism
  • Cell Differentiation / immunology
  • Cells, Cultured
  • Disease Models, Animal
  • Immunoglobulin Subunits / genetics
  • Immunoglobulin Subunits / immunology
  • Lymphocyte Activation / genetics
  • Lymphocyte Activation / immunology
  • Lymphocyte Depletion / methods
  • Mice
  • Mice, Inbred C57BL
  • Mice, Transgenic
  • Superoxide Dismutase / genetics*
  • Superoxide Dismutase-1


  • Autoantibodies
  • Immunoglobulin Subunits
  • Sod1 protein, mouse
  • Superoxide Dismutase
  • Superoxide Dismutase-1