{Reference Type}: Journal Article
{Title}: The biophysical and biochemical properties of the autoimmune regulator (AIRE) protein.
{Author}: Perniola R;Musco G;
{Journal}: Biochim Biophys Acta
{Volume}: 1842
{Issue}: 2
{Year}: Feb 2014
暂无{DOI}: 10.1016/j.bbadis.2013.11.020
{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.