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Fig. 1 | Italian Journal of Pediatrics

Fig. 1

From: Novel insight into Chronic Inflammatory Demyelinating Polineuropathy in APECED syndrome: molecular mechanisms and clinical implications in children

Fig. 1

a and b Aire promotes expression of self-antigens in mTECs and deletion of self-reactive T cells within the thymus. a In a normal thymus, Autoimmune Regulator (Aire) upregulates expression of a large number of tissue-specific self-antigens (Self-Ag) within medullary thymic epithelial cells (mTECs). Developing T cells which recognize these self-antigens with high affinity are eliminated to prevent these T cells from causing autoimmunity. b In Aire deficient individuals, lack of Aire expression within mTECs results in decreased expression of tissue-specific self-antigens in mTECs. Lack of presentation of these self-antigens to self-reactive T cells allows escape of these self-reactive T cells from clonal deletion. Release of self-reactive T cells into the periphery promotes autoimmunity

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