Fezf1 is a novel autoimmune regulator.

The most classically described autoimmune regulator is AIRE, which activates transcription of genes known as tissue-restricted antigens (TRA), like insulin and tyrosine hydroxylase, in a process known as promiscuous gene expression (PGE). TRA activation in the cortical cells of the medulla of the thymus (mTEC) functions to present “self” to developing lymphocytes (B and T cells) for negative selection of autoreactive cells during lymphocyte development. We showed that MHC-II is required for this process. We demonstrate here that the zinc finger nuclear factor Fezf1 functions as an autoimmune regulator, controlling the expression of select TRA as well as a group of genes functioning in tolerance and self non-self recognition in the immune system.