The biophysical and biochemical properties of the autoimmune regulator (AIRE) protein.
| Autor: | Perniola R; Department of Pediatrics - Neonatal Intensive Care, V. Fazzi Regional Hospital, Piazza F. Muratore, I-73100, Lecce, Italy. Electronic address: rperniola@hotmail.com., Musco G; Biomolecular NMR Laboratory, Center of Translational Genomics and Bioinformatics, Dulbecco Telethon Institute at San Raffaele Scientific Institute, Via Olgettina 58, I-20132, Milan, Italy. Electronic address: musco.giovanna@hsr.it. |
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| Jazyk: | angličtina |
| Zdroj: | Biochimica et biophysica acta [Biochim Biophys Acta] 2014 Feb; Vol. 1842 (2), pp. 326-37. Date of Electronic Publication: 2013 Nov 22. |
| DOI: | 10.1016/j.bbadis.2013.11.020 |
| Abstrakt: | 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. (Copyright © 2013 The Authors. Published by Elsevier B.V. All rights reserved.) |
| Databáze: | MEDLINE |
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