X-linked immunodeficiencies: clues to genes involved in T- and B-cell differentiation.
There are five major human X-linked immunodeficiencies, each with a characteristic impairment of T-and/or B-cell differentiation. The molecular bases of these diseases remain unknown but, as Geneviève de Saint Basile and Alain Fischer report, major steps towards that goal have been taken: the location of the defective genes has been precisely defined and the cell lineages and stages of differentiation in which the genes are expressed have been partly identified.
Unit 132, Institut National de la Santé et de la Recherche Médicale, Hôpital Necker-Enfants Malades, Paris, France.
G
G
de Saint Basile
A
A
Fischer
D000818:Animals; D001402:B-Lymphocytes / Q000276:immunology* / Q000473:pathology; D002454:Cell Differentiation; D002874:Chromosome Mapping; D004195:Disease Models, Animal; D004283:Dog Diseases / Q000235:genetics / Q000276:immunology / Q000473:pathology; D004285:Dogs / Q000235:genetics / Q000276:immunology; D004303:Dosage Compensation, Genetic; D005260:Female; D005786:Gene Expression Regulation; D008040:Genetic Linkage; D005819:Genetic Markers; D006579:Heterozygote; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000235:genetics* / Q000276:immunology / Q000473:pathology / Q000662:veterinary; D008297:Male; D051379:Mice; D008817:Mice, Mutant Strains / Q000235:genetics / Q000276:immunology; D010641:Phenotype; D012150:Polymorphism, Restriction Fragment Length; D012376:Rodent Diseases / Q000235:genetics / Q000276:immunology / Q000473:pathology; D013601:T-Lymphocytes / Q000276:immunology* / Q000473:pathology; D014960:X Chromosome
D005819:Genetic Markers