title: Naturally occurring primary deficiencies of the immune system.
abstract: Naturally occurring genetic disorders of the immune system provide many models for the study of its development and function. In a way, their analysis complements the information provided by the generation of genetic defects in mice created using homologous recombination techniques. In this review, the recent findings made in three areas are focused upon deficiencies in T cell differentiation and in T lymphocyte activation, and on the control process of peripheral immune response.
authors.affiliation: Unité INSERM U 429, Hôpital Necker-Enfants Malades, Paris, France.
authors.forename: A
authors.initials: A
authors.lastname: Fischer
authors.forename: M
authors.initials: M
authors.lastname: Cavazzana-Calvo
authors.forename: G
authors.initials: G
authors.lastname: De Saint Basile
authors.forename: J P
authors.initials: JP
authors.lastname: DeVillartay
authors.forename: J P
authors.initials: JP
authors.lastname: Di Santo
authors.forename: C
authors.initials: C
authors.lastname: Hivroz
authors.forename: F
authors.initials: F
authors.lastname: Rieux-Laucat
authors.forename: F
authors.initials: F
authors.lastname: Le Deist
mesh_terms: D000818:Animals; D004195:Disease Models, Animal; D005260:Female; D008040:Genetic Linkage; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000209:etiology / Q000235:genetics* / Q000276:immunology; D007694:Killer Cells, Natural / Q000276:immunology; D008213:Lymphocyte Activation / Q000235:genetics; D008297:Male; D051379:Mice; D016511:Severe Combined Immunodeficiency / Q000235:genetics; D013601:T-Lymphocytes / Q000276:immunology; D014960:X Chromosome / Q000235:genetics; D019014:fas Receptor / Q000235:genetics
chemical_list: D019014:fas Receptor