Autoimmune lymphoproliferative syndromes (ALPS): models for the study of peripheral tolerance.
Lymphocyte cell death is a key event in the homeostasis of the immune system. Lymphocytes can be induced to die because of exposure to toxic agents, because of cytokine withdrawal or because specific cell surface receptors are engaged by their ligands. A number of such receptors belonging to the TNF receptor have been described in the recent past. Among these, the role of the Fas ligand/receptor interaction in the induction of lymphocyte cell death has been enlightened by the study of natural mutants, first described in mouse strains, then in humans. This review discusses the main findings provided by murine studies and clinical observations.
Fischer
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H pital Necker - INSERM U429, Paris, France. fischer@necker.fr
Rieux-Laucat
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Le Deist
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D000818:Animals; D011508:Chondroitin Sulfate Proteoglycans / Q000276:immunology; D004195:Disease Models, Animal; D016326:Extracellular Matrix Proteins / Q000276:immunology; D006801:Humans; D007108:Immune Tolerance / Q000276:immunology*; D008232:Lymphoproliferative Disorders / Q000276:immunology*; D018448:Models, Immunological; D053220:Receptors, TNF-Related Apoptosis-Inducing Ligand; D018124:Receptors, Tumor Necrosis Factor / Q000276:immunology; D019014:fas Receptor / Q000276:immunology
D011508:Chondroitin Sulfate Proteoglycans; C082019:DPT protein, human; D016326:Extracellular Matrix Proteins; D053220:Receptors, TNF-Related Apoptosis-Inducing Ligand; D018124:Receptors, Tumor Necrosis Factor; C105190:TNFRSF10A protein, human; D019014:fas Receptor