First Author | Peng SL | Year | 1998 |
Journal | J Immunol | Volume | 160 |
Issue | 2 | Pages | 652-60 |
PubMed ID | 9551899 | Mgi Jnum | J:45170 |
Mgi Id | MGI:1194483 | Doi | 10.4049/jimmunol.160.2.652 |
Citation | Peng SL, et al. (1998) Perforin protects against autoimmunity in lupus-prone mice. J Immunol 160(2):652-60 |
abstractText | The roles of cytolytic regulatory mechanisms in the immune system of lupus-prone mice were examined in perforin-deficient animals bearing functional or defective (lpr) Fas Ag (CD95). Perforin-deficient Fas+ animals developed accelerated autoimmunity, characterized by increased hypergamma-globulinemia, autoantibody production, and immune deposit-related end-organ disease compared with perforin-intact counterparts. In comparison, perforin-deficient lpr animals had accelerated mortality compared with perforin-intact lpr mice, associated with the abnormal accumulation of CD3+CD4-CD8- alphabeta T cells in conjunction with unaltered hypergammaglobulinemia, autoantibody production, and immune complex renal disease. These results indicate that cytolytic lymphoid regulation plays critical roles in the immune homeostasis of lupus-prone animals, and identify perforin-mediated cytotoxicity as a specific mechanism in the regulation of systemic autoimmunity. |