TY - JOUR
T1 - Experimental myasthenia gravis in Aire-deficient mice
T2 - A link between Aire and regulatory T cells
AU - Aricha, Revital
AU - Feferman, Tali
AU - Berrih-Aknin, Sonia
AU - Fuchs, Sara
AU - Souroujon, Miriam C.
PY - 2012/12
Y1 - 2012/12
N2 - 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.
AB - 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.
KW - Aire
KW - Experimental autoimmune myasthenia gravis (EAMG)
KW - Regulatory T cells
KW - Spleen
KW - Thymus
UR - http://www.scopus.com/inward/record.url?scp=84871556815&partnerID=8YFLogxK
U2 - 10.1111/j.1749-6632.2012.06843.x
DO - 10.1111/j.1749-6632.2012.06843.x
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C2 - 23278585
AN - SCOPUS:84871556815
SN - 0077-8923
VL - 1275
SP - 107
EP - 113
JO - Annals of the New York Academy of Sciences
JF - Annals of the New York Academy of Sciences
IS - 1
ER -