Experimental myasthenia gravis in Aire-deficient mice: A link between Aire and regulatory T cells

Revital Aricha, Tali Feferman, Sonia Berrih-Aknin, Sara Fuchs, Miriam C. Souroujon

Research output: Contribution to journalArticlepeer-review


Aire (autoimmune regulator) has a key role in the establishment of tolerance to autoantigens. Aire-/- mice present decreased thymic expression of AChR, significantly lower frequencies of regulatory T (Treg) cells, and higher expression of Th17 markers, compared to controls. We therefore predicted that Aire-/- mice would be more susceptible to induction of experimental autoimmune myasthenia gravis (EAMG). However, when EAMG was induced in young mice, Aire-/- mice presented a milder disease that wild-type (WT) controls. In contrast, when EAMG was induced in older mice, Aire-/- mice were more severely affected than WT mice. The relative resistance to EAMG in young Aire-/- mice correlated with increased numbers of Treg cells in their spleens compared to young controls. A similar age-related susceptibility was also observed when EAE was induced in Aire-/- mice, suggesting an age-related link among Aire, disease susceptibility, and peripheral Treg cells that may be a general feature of autoimmunity.

Original languageEnglish
Pages (from-to)107-113
Number of pages7
JournalAnnals of the New York Academy of Sciences
Issue number1
StatePublished - Dec 2012


  • Aire
  • Experimental autoimmune myasthenia gravis (EAMG)
  • Regulatory T cells
  • Spleen
  • Thymus


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