| First Author | Anderson MS | Year | 2002 |
| Journal | Science | Volume | 298 |
| Issue | 5597 | Pages | 1395-401 |
| PubMed ID | 12376594 | Mgi Jnum | J:83422 |
| Mgi Id | MGI:2661871 | Doi | 10.1126/science.1075958 |
| Citation | Anderson MS, et al. (2002) Projection of an immunological self shadow within the thymus by the aire protein. Science 298(5597):1395-401 |
| abstractText | 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. |
