What is the function of AIRE?

What is the function of AIRE?

Normal Function The AIRE gene provides instructions for making a protein called the autoimmune regulator. This protein is active primarily in the thymus, which is an organ located behind the breastbone that plays an important role in immune system function.

What is the importance of AIRE in T cell tolerance?

Aire regulates the expression of tissue-specific antigens (TSAs) in medullary thymic epithelial cells (mTECs), which play a critical role in the negative selection of autoreactive T cells and the generation of regulatory T cells.

What is the main function of AIRE during lymphocyte development?

Aire is expressed in the nucleus of mTECs where its primary function is to upregulate the expression of tissue specific antigens (TSAs).

What is AIRE in central tolerance?

An essential molecule in the induction of central tolerance is Autoimmune Regulator (Aire). The AIRE gene was identified by positional cloning of a locus linked to a rare disease, Autoimmune-Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy (APECED) (2, 3).

Are thymocytes T cells?

A Thymocyte is an immune cell present in the thymus, before it undergoes transformation into a T cell. Thymocytes are produced as stem cells in the bone marrow and reach the thymus via the blood.

Is AIRE expressed in bone marrow?

Extrathymic Aire-expressing cells are a distinct bone marrow-derived population that induce functional inactivation of CD4⁺ T cells. Immunity.

What is positive selection of T cells?

Positive selection occurs when double positive T cells bind cortical epithelial cells expressing Class I or Class II MHC plus self peptides with a high enough affinity to get the survival signal.

How do T cells become CD4 or CD8?

Positive selection also determines whether the T cell will become a helper or a cytotoxic T cell. Positive selection on Class I MHC will produce a CD8 Tc cell, while positive selection on Class II MHC will yield a CD4 Th cell.

What are antigen presenting cells?

Antigen-presenting cells (APCs) are a heterogeneous group of immune cells that mediate the cellular immune response by processing and presenting antigens for recognition by certain lymphocytes such as T cells. Classical APCs include dendritic cells, macrophages, Langerhans cells and B cells.

What is positive and negative selection for T cells?

In positive selection, T cells in the thymus that bind moderately to MHC complexes receive survival signals (middle). However, T cells whose TCRs bind too strongly to MHC complexes, and will likely be self-reactive, are killed in the process of negative selection (bottom).

What is plasma in cell?

When separated from the rest of the blood, plasma is a light yellow liquid. Plasma carries water, salts and enzymes. The main role of plasma is to take nutrients, hormones, and proteins to the parts of the body that need it. Cells also put their waste products into the plasma.

What is the relationship between Aire and T cell development?

Here, we will focus on 3 areas relevant to T cell development and Aire: 1) Aire in the regulation of TSA expression and negative selection of thymocytes, 2) additional Aire functions related to T cell development, and 3) ontogeny of Aire-expressing mTECs.

How does Aire cause negative selection of self-recognizing T cells?

Thus AIRE drives negative selection of self-recognizing T cells. When AIRE is defective, T cells that recognize antigens normally produced by the body can exit the thymus and enter circulation. This can result in a variety of autoimmune diseases.

What is the function of Aire?

Transcription factor expressed in the medulla (inner part) of the thymus. It is part of the mechanism which eliminates self-reactive T cells that would cause autoimmune disease. The autoimmune regulator ( AIRE) is a protein that in humans is encoded by the AIRE gene.

What is the autoimmune regulator (Aire)?

View/Edit Mouse. The autoimmune regulator (AIRE) is a protein that in humans is encoded by the AIRE gene. AIRE is a transcription factor expressed in the medulla (inner part) of the thymus.