Abstract
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.
MeSH terms
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Animals
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B-Lymphocytes / immunology*
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B-Lymphocytes / pathology
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Cell Differentiation
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Chromosome Mapping
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Disease Models, Animal
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Dog Diseases / genetics
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Dog Diseases / immunology
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Dog Diseases / pathology
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Dogs / genetics
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Dogs / immunology
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Dosage Compensation, Genetic
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Female
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Gene Expression Regulation
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Genetic Linkage
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Genetic Markers
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Heterozygote
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Humans
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Immunologic Deficiency Syndromes / genetics*
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Immunologic Deficiency Syndromes / immunology
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Immunologic Deficiency Syndromes / pathology
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Immunologic Deficiency Syndromes / veterinary
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Male
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Mice
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Mice, Mutant Strains / genetics
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Mice, Mutant Strains / immunology
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Phenotype
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Polymorphism, Restriction Fragment Length
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Rodent Diseases / genetics
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Rodent Diseases / immunology
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Rodent Diseases / pathology
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T-Lymphocytes / immunology*
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T-Lymphocytes / pathology
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X Chromosome*