We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Projection of an immunological self shadow within the thymus by the aire protein.
- Authors
Anderson, Mark S; Venanzi, Emily S; Klein, Ludger; Chen, Zhibin; Berzins, Stuart P; Turley, Shannon J; von Boehmer, Harald; Bronson, Roderick; Dierich, Andrée; Benoist, Christophe; Mathis, Diane
- Abstract
Humans expressing a defective form of the transcription factor AIRE (autoimmune regulator) develop multiorgan autoimmune disease. We used aire- deficient mice to test the hypothesis that this transcription factor regulates autoimmunity by promoting the ectopic expression of peripheral tissue- restricted antigens in medullary epithelial cells of the thymus. This hypothesis proved correct. The mutant animals exhibited a defined profile of autoimmune diseases that depended on the absence of aire in stromal cells of the thymus. Aire-deficient thymic medullary epithelial cells showed a specific reduction in ectopic transcription of genes encoding peripheral antigens. These findings highlight the importance of thymically imposed "central" tolerance in controlling autoimmunity.
- Publication
Science (New York, N.Y.), 2002, Vol 298, Issue 5597, p1395
- ISSN
1095-9203
- Publication type
Journal Article
- DOI
10.1126/science.1075958