NATURALLY OCCURRING PRIMARY DEFICIENCIES OF THE IMMUNE SYSTEM

• Published in: Annual review of immunology Vol. 15; no. 1; pp. 93 - 124
• Main Authors: Fischer, A, Cavazzana-Calvo, M, Basile, G. De Saint, DeVillartay, J. P, Di Santo, J. P, Hivroz, C, Rieux-Laucat, F, Le Deist, F
• Format: Journal Article
• Language: English
• Published: Palo Alto, CA 94303-0139 Annual Reviews 01-01-1997; 4139 El Camino Way, P.O. Box 10139 Annual Reviews, Inc; USA
• Subjects: Animals; apoptosis; Disease Models, Animal; fas Receptor - genetics; Female; Genetic Linkage; Humans; Immunologic Deficiency Syndromes - etiology; Immunologic Deficiency Syndromes - genetics; Immunologic Deficiency Syndromes - immunology; Killer Cells, Natural - immunology; Lymphocyte Activation - genetics; Male; Mice; primary immunodeficiencies; regulation; severe combined immunodeficiencies; Severe Combined Immunodeficiency - genetics; T lymphocytes; T-Lymphocytes - immunology; X Chromosome - genetics
• ISSN: 0732-0582; 1545-3278
• DOI: 10.1146/annurev.immunol.15.1.93

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.