Ataxia-Telangiectasia: A Multisystem Hereditary Disease with Immunodeficiency, Impaired Organ Maturation, X-Ray Hypersensitivity, and a High Incidence of Neoplasia
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References (53)
MHC-Restricted Cytotoxic T Cells: Studies on the Biological Role of Polymorphic Major Transplantation Antigens Determining T-Cell Restriction-Specificity, Function, and Responsiveness
1979 • 1,969 citations
Defective Repair Replication of DNA in Xeroderma Pigmentosum
1968 • 1,739 citations
Ataxia telangiectasia: a human mutation with abnormal radiation sensitivity
1975 • 984 citations
Structure of the human immunoglobulin μ locus: Characterization of embryonic and rearranged J and D genes
1981 • 959 citations
Sequences at the somatic recombination sites of immunoglobulin light-chain genes
1979 • 859 citations
Incidence of malignant tumors in U.S. children
1975 • 848 citations
Radiosensitivity in ataxia-telangiectasia: a new explanation.
1980 • 784 citations
Xeroderma Pigmentosum
1974 • 755 citations
ROLE OF SUPPRESSOR T CELLS IN PATHOGENESIS OF COMMON VARIABLE HYPOGAMMAGLOBULINÆMIA
1974 • 717 citations
Ataxia-telangiectasia; a familial syndrome of progressive cerebellar ataxia, oculocutaneous telangiectasia and frequent pulmonary infection.
1958 • 422 citations
Chromosomal location of the genes for human immunoglobulin heavy chains.
1979 • 368 citations
Morphogenetic interactions in the development of the mouse thymus gland
1960 • 336 citations
SERUM-ALPHA-FETOPROTEIN LEVELS IN PATIENTS WITH ATAXIA-TELANGIECTASIA
1972 • 327 citations
ATAXIA-TELANGIECTASIA ITS ASSOCIATION WITH A DEFECTIVE THYMUS, IMMUNOLOGICAL-DEFICIENCY DISEASE, AND MALIGNANCY
1964 • 308 citations
An immunoglobulin heavy-chain gene is formed by at least two recombinational events
1980 • 306 citations
Malignant neoplasms in the families of patients with ataxia-telangiectasia.
1976 • 285 citations
ATAXIA TELANGIECTASIA: An Inherited Human Disorder Involving Hypersensitivity to Ionizing Radiation and Related DNA-Damaging Chemicals
1979 • 254 citations
Human Diseases Associated with Defective DNA Repair
1979 • 224 citations
HUMAN DISORDERS SHOWING INCREASED SENSITIVITY TO THE INDUCTION OF GENETIC DAMAGE
1978 • 212 citations
Identification of ataxia telangiectasia heterozygotes, a cancer prone population
1978 • 211 citations
IgG2 Deficiency in Ataxia-Telangiectasia
1982 • 180 citations
Unusual levels of (ADP-ribose)n and DNA synthesis in ataxia telangiectasia cells following γ-ray irradiation
1980 • 175 citations
Polyclonal B-Cell Activators in the Study of the Regulation of Immunoglobulin Synthesis in the Human System
1982 • 162 citations
Lymphoid tissue abnormalities associated with ataxia-telangiectasia
1966 • 162 citations
A Low Molecular Weight Immunoglobulin Antigenically Related to 19 S IgM*
1967 • 156 citations
Enhanced radiosensitivity of cultured fibroblasts from ataxia telangiectasia heterozygotes manifested by defective colony-forming ability and reduced DNA repair replication after hypoxic gamma-irradiation.
1979 • 156 citations
Immunoglobulin E Deficiency in Ataxia-Telangiectasia
1969 • 150 citations
Unusual sensitivity of ataxia telangiectasia cells to bleomycin.
1979 • 148 citations
Immunoglobulin metabolism in ataxia telangiectasia
1968 • 114 citations
Complementation of the defects of DNA synthesis in irradiated and unirradiated ataxia-telangiectasia cells.
1982 • 112 citations
Genetic heterogeneity in ataxia-telangiectasia studied by cell fusion.
1982 • 110 citations
The rate of DNA synthesis in normal human and ataxia telangiectasia cells after exposure to X-irradiation
1981 • 109 citations
Immunoglobulin E in Immunologic Deficiency Diseases. I. RELATION OF IGE AND IGA TO RESPIRATORY TRACT DISEASE IN ISOLATED IGE DEFICIENCY, IGA DEFICIENCY, AND ATAXIA TELANGIECTASIA
1972 • 103 citations
Spontaneous cytogenetic abnormalities in lymphocytes from thirteen patients with ataxia telangiectasia
1981 • 103 citations
Ataxia-telangiectasia: some historic, clinical and pathologic observations.
1975 • 101 citations
RADIOSENSITIVITY IN HUNTINGTON'S DISEASE: IMPLICATIONS FOR PATHOGENESIS AND PRESYMPTOMATIC DIAGNOSIS
1980 • 95 citations
Defect in IgA secretion and in IgA specific suppressor cells in patients with selective IgA deficiency.
1976 • 85 citations
Disorders of B cells and helper T cells in the pathogenesis of the immunoglobulin deficiency of patients with ataxia telangiectasia.
1983 • 83 citations
Immunologic Deficiency in Ataxia Telangiectasia
1965 • 82 citations
Decreased DNA repair synthesis and defective colony-forming ability of ataxia telangiectasia fibroblast cell strains treated with N-methyl-N'-nitro-N-nitrosoguanidine.
1980 • 69 citations
[Ataxiatelangiectasis. (7 personal cases)].
1961 • 68 citations
Hypersensitivity to N-methyl-N'-nitro-N-nitrosoguanidine in fibroblasts from patients with Huntington disease, familial dysautonomia, and other primary neuronal degenerations.
1981 • 68 citations
Virus Specificity of Human Influenza Virus-Immune Cytotoxic T Cells
1979 • 62 citations
Immunologic Studies in Ataxia-Telangiectasia
1966 • 58 citations
Bleomycin-resistant DNA synthesis in ataxia telangiectasia cells
1981 • 45 citations
In vitro generation of antigen-specific hemolytic plaque-forming cells from human peripheral blood mononuclear cells.
1981 • 44 citations
Some DNA-repair-deficient human syndromes and their implications for human health
1981 • 43 citations
Plasma levels of carcinoembryonic antigen in patients with ataxia-telangiectasia
1978 • 28 citations
DNA repair enzyme deficiency and in vitro complementation of the enzyme activity in cell-free extracts from ataxia telangiectasia fibroblasts
1981 • 27 citations
DNA repair in lymphoblastoid cell lines established from human genetic disorders
1980 • 26 citations
Gamma-ray induced inhibition of DNA synthesis in ataxia telangiectasia fibroblasts is a function of excision repair capacity
1980 • 25 citations
Ataxia-telangiectasia in Israel. With observations on its relationship to malignant disease.
1971 • 22 citations
Human cytotoxic T cell responses to trinitrophenyl hapten and influenza virus. Diversity of restriction antigens and specificity of HLA-linked genetic regulation.
1980 • 20 citations