PMID: 11324693Apr 28, 2001Paper

Autoimmune lymphoproliferative syndromes (ALPS): models for the study of peripheral tolerance

Reviews in Immunogenetics
A FischerF Le Deist

Abstract

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.

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Autoimmune lymphoproliferative syndrome (ALPS) is a rare genetic disorder of abnormal lymphocyte survival caused by defective Fas mediated apoptosis. Discover the latest research on ALPS here.