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Publication : Perforin protects against autoimmunity in lupus-prone mice.

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.
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