ATAXIA TELANGIECTASIA: An Inherited Human Disorder Involving Hypersensitivity to Ionizing Radiation and Related DNA-Damaging Chemicals

Paterson, Malcolm C.; Smith, Paul J.

doi:10.1146/annurev.ge.13.120179.001451

Cited by 242 publications

(68 citation statements)

References 39 publications

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“…like all the AT strains tested to date (Arlett and Lehmann, 1978;Paterson and Smith, 1979). The D O values (X-ray doses required to inactivate 63~ of the cell population, estimated from the straight portion of the survival curves) were 150 rad for N1KA, 135 tad for NHF3, 50 tad for ATlaS and 56 rad for AT4BI cells.…”

Section: Resultsmentioning

confidence: 95%

“…Affected individuals also show elevated risk of lymphoreticular malignancy (see reviews by Arlett and Lehman_n, 1978;Paterson and Smith, 1979). In 1975, Taylor et al demonstrated that skin fibroblasts derived from AT patients were about three times more sensitive to the lethal effect of r-rays than fibroblasts from normal donors.…”

Section: Introductionmentioning

confidence: 99%

“…In 1975, Taylor et al demonstrated that skin fibroblasts derived from AT patients were about three times more sensitive to the lethal effect of r-rays than fibroblasts from normal donors. Since then, more than 20 AT cell strains from genetically unrelated patients have been found to be hypersensitive to X-and r-rays (Paterson and Smith, 1979;Arlett and Harcourt, 1980). These results suggested that AT cells might have some defects in repair of DNA damage produced by ionizing radiations, analogous to xeroderma pigmentosum (XP) cells which are defective in repairing DNA damage induced by ultraviolet light (UV) (Cleaver, 1968).…”

Section: Introductionmentioning

confidence: 99%

“…This indicates that AT cells may be defective in a repair exonuclease (primer activating enzyme) which converts some of the r-ray-induced lesions into a substrate suitable for DNA polymerase action. However, rates of rejoining of 7-ray-induced single as well as double strand breaks in DNA, which are the predominant DNA lesions produced by ionizing radiations, were very similar in cells from normal donors and from AT patients (Taylor et aI., 1975;Paterson and Smith, 1979). Recently, a new characteristic was found with many AT strains; the rate of DNA replication after exposure to ionizing radiations was inhibited to a lesser extent in AT cells than in normal cells (Houldsworth and Lavin, 1980;Painter and Young, 1980).…”

Section: Introductionmentioning

confidence: 99%

“…Therefore, analysis of chemical sensitivities of AT cells may provide useful informations as to the nature of the defects underlying in this disease. The studies with chemical sensitivity of AT cells have been reported sporadically in the literatures (Paterson and Smith, 1979), however, comprehensive studies have not been made at least with Japanese AT cell strains.…”

Section: Introductionmentioning

confidence: 99%

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The sensitivities to radiations and radiomimetic chemicals of cells from patients with ataxia telangiectasia

et al. 1983

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SummaryThe lethal action of physical and chemical agents on a fibroblast strain derived from a Japanese patient with ataxia telangiectasia (AT) was measured by the cellular colony forming ability, in comparison with a British AT cell strain. Both of the AT strains showed a significantly increased sensitivity to X-rays and art antitumor agent Neomycin, as compared with normal fibroblasts. Also, the two AT strains were slightly more sensitive to methyl methanesulfonate than normal cells. The British AT strain (AT4BI) exhibited a marked sensitivity to 4-nitroquinoline 1-oxide and to mitomycin C compared with normal cells, whereas the Japanese AT strain (AT1OS) showed normal response to 4-nitroquinoline 1-oxide and a reduced sensitivity to mitomycin C. Sensitivity of AT1OS cells to N-methyl-N'-nitro-N-nitrosoguanidine was also normal under our experimental conditions.

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Section: Resultsmentioning

confidence: 95%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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The sensitivities to radiations and radiomimetic chemicals of cells from patients with ataxia telangiectasia

et al. 1983

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Increased radiosensitivity in a child with T-cell non-Hodgkin's lymphoma

Attard-Montalto¹,

Saha²,

Kingston³

et al. 1996

Med. Pediatr. Oncol.

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Mutagen sensitivity as a predisposing factor in familial oral cancer

et al. 1996

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Pedigree analysis of the oral cancer (OC) patients registered at our Centre had disclosed familial aggregation of oral cancer which hitherto has not been largely reported. There is a paucity of information on the genetic determinism for familial oral cancer predisposition. Therefore, we investigated constitutional chromosome abnormalities and bleomycin-induced chromosome sensitivity of 7 familial and 10 sporadic oral cancer patients and 14 unaffected family members (first-degree relatives) to determine whether these factors could give any clues regarding cancer-predisposing factors. Neither the oral cancer patients nor the unaffected family members showed any constitutional chromosomal abnormalities. However, with regard to bleomycin sensitivity, there was significant difference between the oral-cancer patients and unaffected relatives. The mean b/c value was I .68 f 0.48 for familial OC patients, I. I 2 f 0.36 for sporadic OC patients and 0.52 f 0. I 8 for the unaffected family members (p < 0.00 I). A noteworthy observation was that one unaffected family member also showed bleomycin hypersensitivity and expressed a mean b/c value of I .32, at the initiation of the study. That patient later developed oral carcinoma. This clearly demonstrates that mutagen hypersensitivity among unaffected relatives in OC families may be related to cancer predisposition. The mutagen sensitivity study is being continued in a larger series of subjects, for the development of a cytogenetic marker for prediction of cancer susceptibility.8 1996 Wiley-Liss, Inc.

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ATAXIA TELANGIECTASIA: An Inherited Human Disorder Involving Hypersensitivity to Ionizing Radiation and Related DNA-Damaging Chemicals

Cited by 242 publications

References 39 publications

The sensitivities to radiations and radiomimetic chemicals of cells from patients with ataxia telangiectasia

The sensitivities to radiations and radiomimetic chemicals of cells from patients with ataxia telangiectasia

Increased radiosensitivity in a child with T-cell non-Hodgkin's lymphoma

Mutagen sensitivity as a predisposing factor in familial oral cancer

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