AIRE or Autoimmune Regulator is a putative transcription factor expressed primarily in mTECs that is believed to regulate transcription of many peripheral self-antigens.
Absence of AIRE in human patients results in autoimmune polyendocrinopahy-candidiasis-ectodermal dystrophy. AIRE -/- mice exhibit a defined profile of autoimmune diseases.
Lymphotoxin signaling is necessary for expression or Aire and its downstream target genes