Perforin protects against autoimmunity in lupus-prone mice.

Peng SL, Moslehi J, Robert ME, Craft J
J Immunol. 1998 160 (2): 652-60

PMID: 9551899

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 hypergammaglobulinemia, 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.

MeSH Terms (16)

Animals Autoimmune Diseases Cell Movement Crosses, Genetic Disease Susceptibility fas Receptor Lupus Nephritis Lymphocytes Membrane Glycoproteins Mice Mice, Inbred C57BL Mice, Inbred MRL lpr Mice, Knockout Perforin Pore Forming Cytotoxic Proteins Survival Analysis

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