What is the Origin of Autoimmune Regulator (AIRE)?

The term and concept originated in molecular immunology and genetics following the identification and cloning of the AIRE gene in the mid-1990s. Its discovery was directly linked to the investigation of Autoimmune Polyendocrinopathy-Candidiasis-Ectodermal Dystrophy, a rare genetic syndrome characterized by simultaneous failure of multiple endocrine glands. The protein's name reflects its primary, regulatory role in preventing self-directed immune responses.

What is the Mechanism of Autoimmune Regulator (AIRE)?

AIRE operates by inducing the "promiscuous gene expression" of tissue-specific antigens, such as insulin or thyroid peroxidase, within the medullary thymic epithelial cells. This process exposes developing T-cells to a broad spectrum of the body's own proteins, essentially creating an immunological self-shadow. T-cells that bind strongly to these self-antigens are subsequently eliminated through apoptosis, a process known as negative selection, thereby safeguarding peripheral endocrine tissues from T-cell mediated autoimmune attack.

Autoimmune Regulator (AIRE) ∞ Area

Autoimmune Regulator (AIRE)

Meaning

Autoimmune Regulator, or AIRE, is a pivotal protein encoded by the AIRE gene, functioning as a transcription factor primarily within the thymus gland. This protein is essential for establishing central immunological tolerance, which is the body’s mechanism for teaching T-cells to recognize and ignore its own proteins. Dysfunction of this regulator is directly implicated in the failure of self-recognition, leading to various polyendocrine autoimmune syndromes. It acts as a critical checkpoint in immune cell maturation, preventing the release of cells that would otherwise attack the body’s tissues.