LEUKqMIA AND LYMPHOCYTES IN ATAXIA-TELANGIECTASIA

Hecht, Frederick; Koler, RobertD.; Rigas, DemetriosA.; Dahnke, GretaS.; Case, MiltonP.; Tisdale, Virginia V.; Miller, RobertW.

doi:10.1016/s0140-6736(66)90517-4

Cited by 187 publications

(25 citation statements)

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“…This association between cytogenetic imbalance and neoplasia, although not necessarily causal, apparently obtains whether it is due to an inherited tendency [7,17,31] or induced by exogenous agents [6,14].…”

Section: Discussionmentioning

confidence: 99%

The Effect of LSD-25 on the Chromosomes of Children Exposed in Utero

et al. 1968

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ExtractThis paper reports the cytogenetic investigation of nine children exposed to LSD-25 ,in utero, four children of'other LSD users not exposed in utero, the mothers of these children, and six additional adults taking the drug. In comparison with matched controls, significantly elevated frequencies of chromosomal damage were observed in all the individuals exposed. The frequencies of aberrations, however, were markedly reduced in those children not exposed in utero. Eight structural rearrangements were observed, six dicentric chromosomes and two quadriradial formations, among those individuals exposed to the drug, but none was observed in the controls. In spite of obvious chromosomal aberrations, all of the individuals in this study were apparently healthy and showed no obvious birth defects. Speculation

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Section: Discussionmentioning

confidence: 99%

The Effect of LSD-25 on the Chromosomes of Children Exposed in Utero

et al. 1968

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“…Ataxia telangiectasia (AT) is a human autosomal recessive disorder characterized clinically by progressive cerebellar degeneration, ocular telangiectasia, immunodeficiency and proness to cancer [8,20,24,25]. Patients with AT are unusually sensitive to ionizing radiation, and cultured cells derived from AT patients show an increased sensitivity to ionizing radiation [19,26].…”

Section: Culture Of the Cells And X-irradiationmentioning

confidence: 99%

“…We have reported that the hypersensitivity of LEC rats to whole-body irradiation is controlled by a single autosomal recessive gene, xhs [6], and that the frequencies of all types of chromosome aberrations induced by X-irradiation in the bone marrow cells of LEC rats are approximately 2-to 3-fold higher than those of WKAH rats [17]. Recently, we reported that the fibroblast cell lines established from LEC rats show a high sensitivity to Xirradiation and that the DNA synthesis is resistant to X-irradiation in LEC rat cells [7].Ataxia telangiectasia (AT) is a human autosomal recessive disorder characterized clinically by progressive cerebellar degeneration, ocular telangiectasia, immunodeficiency and proness to cancer [8,20,24,25]. Patients with AT are unusually sensitive to ionizing radiation, and cultured cells derived from AT patients show an increased sensitivity to ionizing radiation [19,26].…”

mentioning

confidence: 99%

Abnormal G1 Arrest in the Cell Lines from LEC Strain Rats after X-Irradiation.

Hayashi

Uehara

Kirisawa

et al. 1997

The Journal of Veterinary Medical Science

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ABSTRACT. The effect of X-irradiation of cell lines from LEC and WKAH strain rats on a progression of cell cycle was investigated. When WKAH rat cells were exposed to 5 Gy of X-rays and their cell cycle distribution was determined by a flow cytometer, the proportion of Sphase cells decreased and that of G2/M-phase cells increased at 8 hr post-irradiation. At 18 and 24 hr post-irradiation, approximately 80% of the cells appeared in the G1 phase. On the contrary, the proportion of S-phase cells increased and that of G1-phase cells decreased in LEC rats during 8-24 hr post-irradiation, compared with that at 0 hr post-irradiation. Thus, radiation-induced delay in the progression from the G1 phase to S phase (G1 arrest) was observed in WKAH rat cells but not in LEC rat cells. In the case of WKAH rat cells, the intensities of the bands of p53 protein increased at 1 and 2 hr after X-irradiation at 5 Gy, compared with those of unirradiated cells and at 0 hr post-irradiation. In contrast, the intensities of the bands were faint and did not significantly increase in LEC rat cells during 0-6 hr incubation after X-irradiation. Present results suggested that the radioresistant DNA synthesis in LEC rat cells is thought to be due to the abnormal G1 arrest following X-irradiation. -KEY WORDS: cell cycle, G1 arrest, LEC strain rat, p53 protein, X-irradiation.J. Vet. Med. Sci. 59(9): 769-773, 1997 to S phase in the cell lines derived from lung fibroblasts of LEC rats and showed an abnormal G1 arrest following Xirradiation. MATERIALS AND METHODS Culture of the cells and X-irradiation:The rat fibroblast cell lines were established from lungs of LEC and WKAH rats by SV 40 immortalization as described previously [7]. Cells were grown in monolayer culture in Eagle's minimum essential medium (MEM) containing 10% fetal calf serum (FCS). Cell cultures were kept at ambient humidity and 37°C in an atmosphere containing 5% CO 2 .X-irradiation was carried out utilizing a Hitachi MBR-1520R X-ray generator operated at 150 kV and 15 mA with a 0.5 mm Cu + 1.0 mm Al filter at a dose rate of 0.95 Gy/ min.Flow cytometry: The cell cycle phase distribution of a cell population was determined by measuring the DNA content of individual cells by flow cytometry. In preparation for flow cytometry, cells (1 × 10 6 to 5 × 10 6 ) were exposed to 5 Gy of X-rays. After X-irradiation, the cells were incubated at 37°C for 0-24 hr, harvested by trypsinization, washed with PBS and then pelleted by centrifugation at 500 × g. The cells were fixed in 5 ml of cold 70% ethanol for 30 min at room temperature and then stored at 20°C. Just prior to flow cytometric analysis, individual samples were treated with RNase (1 mg/ml) for 30 min at room temperature and then stained with propidium iodide according to the manufacturer's instructions for the cellular DNA flow cytometric analysis reagent set (Boeringer Mannheim). Fluorescence was measured with a Coulter EPICS Elite flow sorter using a 610-nm filter. Cell cycle distribution was determined using multicycle software. ...

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“…[1] mettaient l'accent sur l'instabilité chromosomique observée dans les cellules des patients AT. Cette instabilité chromosomique, caracté risée par une augmentation de la fréquence des cassures, lacunes, images radiales, etc.…”

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Aspects cytogénétiques de l'ataxie télangiectasie

Aurias¹

1986

Med Sci (Paris)

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LEUKqMIA AND LYMPHOCYTES IN ATAXIA-TELANGIECTASIA

Cited by 187 publications

References 0 publications

The Effect of LSD-25 on the Chromosomes of Children Exposed in Utero

The Effect of LSD-25 on the Chromosomes of Children Exposed in Utero

Abnormal G1 Arrest in the Cell Lines from LEC Strain Rats after X-Irradiation.

Aspects cytogénétiques de l'ataxie télangiectasie

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