title: Experimental myasthenia gravis in Aire-deficient mice: a link between Aire and regulatory T cells.
abstract: 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 (T(reg)) 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 T(reg) 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 T(reg) cells that may be a general feature of autoimmunity.
authors.affiliation: Department of Immunology, The Weizmann Institute of Science, Rehovot, Israel.
authors.forename: Revital
authors.initials: R
authors.lastname: Aricha
authors.forename: Tali
authors.initials: T
authors.lastname: Feferman
authors.forename: Sonia
authors.initials: S
authors.lastname: Berrih-Aknin
authors.forename: Sara
authors.initials: S
authors.lastname: Fuchs
authors.forename: Miriam C
authors.initials: MC
authors.lastname: Souroujon
mesh_terms: D000375:Aging; D000818:Animals; D020022:Genetic Predisposition to Disease; D051379:Mice; D018345:Mice, Knockout; D009157:Myasthenia Gravis / Q000276:immunology*; D050378:T-Lymphocytes, Regulatory / Q000276:immunology*; D014157:Transcription Factors / Q000235:genetics*; D000096927:AIRE Protein
chemical_list: D014157:Transcription Factors; D000096927:AIRE Protein