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AIRE is a transcription factor expressed in the medulla [broken anchor] (inner part) of the thymus. It is part of the mechanism which eliminates self-reactive T cells that would cause autoimmune disease. It exposes T cells to normal, healthy proteins from all parts of the body, and T cells that react to those proteins are destroyed.
In 1989, two scientific groups came up with the hypothesis that the thymus expresses genes which are in the periphery, strictly expressed by specific tissues (e.g.: Insulin produced by β cells of the pancreas) to subsequently present these so-called "tissue-restricted antigens" (TRAs) from almost all parts of the body to developing T cells in order to test which TCRs recognize self-tissues ...
Aire mediates negative selection of auto-reactive T-cells and organ-specific antigens' expression on mTECs. The outcome of a single gene mutation in the autoimmune regulator Aire is systematic disease APECED (APS-1), which is manifested by mucocutaneous candidiasis, hypoparathyroidism and adrenal insufficiency.
T cells are one of the important types of white blood cells of the immune system and play a central role in the adaptive immune response. T cells can be distinguished from other lymphocytes by the presence of a T-cell receptor (TCR) on their cell surface. T cells are born from hematopoietic stem cells, [1] found in the bone marrow.
Major function of cTECs is to positively select those T cells that are capable to recognize and interact with MHC molecules on their surface [3]. Once T cell precursors enter the thymic cortex, they start their transformation from double negative stages (T cell without surface expression of CD4 and CD8 co-receptors) to a double positive stage (T cell with surface expression of both co ...
The important role of the AIRE gene, and the role of negative selection in preventing autoreactive T cells from maturing, was understood by 1994. [ 14 ] Recently, advances in immunology have allowed the function of the thymus in T-cell maturation to be more fully understood.
The regulatory T cells (Tregs / ˈ t iː r ɛ ɡ / or T reg cells), formerly known as suppressor T cells, are a subpopulation of T cells that modulate the immune system, maintain tolerance to self-antigens, and prevent autoimmune disease. T reg cells are immunosuppressive and generally suppress or downregulate induction and proliferation of ...
An autoimmune response is prevented by eliminating cells that self-react. For T cells, this occurs in the thymus by testing the cell against an array of self antigens expressed through the function of the autoimmune regulator (AIRE). The immunoglobulin lambda light chain locus contains protein-coding genes that can be lost with its rearrangement.