Hypersensitivity to ionizing radiation, in vitro, in a new chromosomal breakage disorder, the Nijmegen Breakage syndrome
Autor: | T.W.J. Hustinx, J. de Wit, J. M. J. C. Scheres, Nicolaas G. J. Jaspers, R. D. F. M. Taalman |
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Rok vydání: | 1983 |
Předmět: |
DNA Replication
Male Adolescent DNA Repair Cell Survival Biology Toxicology Bleomycin Chromosomes Ionizing radiation chemistry.chemical_compound Breakage Chromosome instability Genetics medicine Humans Molecular Biology Chromosome Aberrations DNA synthesis Chromosome Fragility X-Rays food and beverages Syndrome medicine.disease Molecular biology In vitro chemistry embryonic structures Ataxia-telangiectasia Nijmegen breakage syndrome |
Zdroj: | Mutation Research/DNA Repair Reports. 112:23-32 |
ISSN: | 0167-8817 |
DOI: | 10.1016/0167-8817(83)90021-4 |
Popis: | The Nijmegen Breakage Syndrome (NBS) is a new chromosomal instability disorder different from ataxia telangiectasia (AT) and other chromosome-breakage syndromes. Cells from an NBS patient appeared hypersensitive to X-irradiation. X-rays induced significantly more chromosomal damage in NBS lymphocytes and fibroblasts than in normal cells. The difference was most pronounced after irradiation in G2. Further, NBS fibroblats were more readily killed by X-rays than normal fibroblasts. In addition, the DNA synthesis in NBS cells was more resistant to X-rays and bleomycin than that in normal cells. The reaction of NBS cells to X-rays and bleomycin was similar to that of cells from patients with ataxia telangiectasia. Our results indicate that NBS and AT, which also have similar chromosomal characteristics, must be closely related. |
Databáze: | OpenAIRE |
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