NATURALLY OCCURRING PRIMARY DEFICIENCIES OF THE IMMUNE SYSTEM

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,...

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Bibliographic Details
Published in:Annual review of immunology 1997-01, Vol.15 (1), p.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: Article
Language:English
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
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Summary: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.
ISSN:0732-0582
1545-3278
DOI:10.1146/annurev.immunol.15.1.93