Journal of Neurology

, Volume 256, Issue 8, pp 1228–1235

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

  • Shulamit Naor
  • Zohar Keren
  • Tomer Bronshtein
  • Efrat Goren
  • Marcelle Machluf
  • Doron Melamed
Original Communication

DOI: 10.1007/s00415-009-5097-3

Cite this article as:
Naor, S., Keren, Z., Bronshtein, T. et al. J Neurol (2009) 256: 1228. doi:10.1007/s00415-009-5097-3

Abstract

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 Igμ-deficient mice (μMT), 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.

Keywords

Amyotrophic lateral sclerosis (ALS)B lymphocytesAntibodiesAutoimmunity

Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Shulamit Naor
    • 1
  • Zohar Keren
    • 1
  • Tomer Bronshtein
    • 2
  • Efrat Goren
    • 2
  • Marcelle Machluf
    • 2
  • Doron Melamed
    • 1
    • 3
  1. 1.Department of Immunology, Bruce Rappaport Faculty of MedicineTechnion-Israel Institute of TechnologyHaifaIsrael
  2. 2.Faculty of Food Engineering and BiotechnologyTechnion-Israel Institute of TechnologyHaifaIsrael
  3. 3.Bruce Rappaport Faculty of Medicine and Rappaport Family Institute for Research in the Medical SciencesTechnion-Israel Institute of TechnologyHaifaIsrael