| First Author | Liston A | Year | 2003 |
| Journal | Nat Immunol | Volume | 4 |
| Issue | 4 | Pages | 350-4 |
| PubMed ID | 12612579 | Mgi Jnum | J:128567 |
| Mgi Id | MGI:3767499 | Doi | 10.1038/ni906 |
| Citation | Liston A, et al. (2003) Aire regulates negative selection of organ-specific T cells. Nat Immunol 4(4):350-4 |
| abstractText | Autoimmune polyendocrinopathy syndrome type 1 is a recessive Mendelian disorder resulting from mutations in a novel gene, AIRE, and is characterized by a spectrum of organ-specific autoimmune diseases. It is not known what tolerance mechanisms are defective as a result of AIRE mutation. By tracing the fate of autoreactive CD4+ T cells with high affinity for a pancreatic antigen in transgenic mice with an Aire mutation, we show here that Aire deficiency causes almost complete failure to delete the organ-specific cells in the thymus. These results indicate that autoimmune polyendocrinopathy syndrome 1 is caused by failure of a specialized mechanism for deleting forbidden T cell clones, establishing a central role for this tolerance mechanism. |
