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Ataxia-Telangiectasia: A Multisystem Hereditary Disease with Immunodeficiency, Impaired Organ Maturation, X-Ray Hypersensitivity, and a High Incidence of Neoplasia

Data up to Jan 2025

Published1983
Citations103
References53

Total Citations Per Year

Abstract

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

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Ataxia-Telangiectasia: A Multisystem Hereditary Disease with Immunodeficiency, Impaired… (1983) – Annals of Internal Medicine | Metascience Observatory Explorer