Research ArticleAUTOIMMUNITY

AIRE expression controls the peripheral selection of autoreactive B cells

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Science Immunology  12 Apr 2019:
Vol. 4, Issue 34, eaav6778
DOI: 10.1126/sciimmunol.aav6778

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AIRE and autoreactivity

Autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy (APECED) is a condition caused by mutations in the autoimmune regulator (AIRE) gene and is associated with central T cell tolerance defects and circulating autoantibodies. Sng et al. now observe that T cell development altered by AIRE deficiency is associated with a greater frequency of autoreactive mature naïve B cells in the periphery. This defect in peripheral B cell tolerance is linked to fewer regulatory T cells that did not include common TCRβ clones found instead in the conventional T cell compartment. This T cell defect may not prevent the selection and expansion of autoreactive B cells to peripheral self-antigens, thus leading to autoantibody production.