The biophysical and biochemical properties of the autoimmune regulator (AIRE) protein

Roberto Perniola, Giovanna Musco

Research output: Contribution to journalArticle

Abstract

AIRE (for autoimmune regulator) is a multidomain protein that performs a fundamental function in the thymus and possibly in the secondary lymphoid organs: the regulation, especially in the sense of activation, of the process of gene transcription in cell lines deputed to the presentation of self-antigens to the maturing T lymphocytes. The apoptosis of the elements bearing T-cell receptors with critical affinity for the exhibited self-antigens prevents the escape of autoreactive clones and represents a simple and efficient mechanism of deletional self-tolerance. However, AIRE action relies on an articulated complex of biophysical and biochemical properties, in most cases attributable to single subspecialized domains. Here a thorough review of the matter is presented, with a privileged look at the pathogenic changes of AIRE that interfere with such properties and lead to the impairment in its chief function.

Original languageEnglish
Pages (from-to)326-337
Number of pages12
JournalBiochimica et Biophysica Acta - Molecular Basis of Disease
Volume1842
Issue number2
DOIs
Publication statusPublished - Feb 2014

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Keywords

  • Autoimmune polyendocrinopathies
  • Post-translational protein processing
  • Protein conformation
  • Self-tolerance
  • Transcription factors

ASJC Scopus subject areas

  • Molecular Biology
  • Molecular Medicine

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