Characterization of cell cycle checkpoint responses after ionizing radiation in Nijmegen breakage syndrome cells

Nijmegen breakage syndrome (NBS), which in the past also has been classified as a variant of ataxia telangiectasia (AT), is characterized by cancer proneness and extreme sensitivity to ionizing radiation. We investigated the DNA damage responses of four independent primary NBS fibroblast cell lines....

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Bibliographic Details
Published in:Cancer research (Chicago, Ill.) Ill.), 1998-06, Vol.58 (11), p.2316-2322
Main Authors: YAMAZAKI, V, WEGNER, R.-D, KIRCHGESSNER, C. U
Format: Article
Language:English
Subjects:
Abnormalities, Multiple - genetics
Ataxia Telangiectasia - genetics
Biological and medical sciences
Cell Cycle - radiation effects
Cells, Cultured
Chromosome Breakage
Fibroblasts - cytology
Fibroblasts - radiation effects
Flow Cytometry
G1 Phase - radiation effects
G2 Phase - radiation effects
Humans
Medical sciences
Microcephaly - genetics
Radiation therapy and radiosensitizing agent
Radiation Tolerance - genetics
Syndrome
Treatment with physical agents
Treatment. General aspects
Tumor Suppressor Protein p53 - metabolism
Tumor Suppressor Protein p53 - radiation effects
Tumors
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Summary:Nijmegen breakage syndrome (NBS), which in the past also has been classified as a variant of ataxia telangiectasia (AT), is characterized by cancer proneness and extreme sensitivity to ionizing radiation. We investigated the DNA damage responses of four independent primary NBS fibroblast cell lines. Following a low dose of ionizing radiation, p53 is mostly induced with slower kinetics and shows more transient induction in NBS fibroblasts. Nonetheless, this damage-induced protein appears biologically functional: unsynchronized and synchronized NBS cells show a G1 arrest after ionizing radiation as determined by bivariate flow cytometry. Neither an AT cell line nor a NBS cell line transformed with human papillomavirus genes E6 and E7 shows a G1 arrest. Furthermore, NBS cells show a normal G2 block, unlike that shown for AT cells. These data provide a cellular distinction between NBS and AT, thereby clearly separating the NBS from the AT syndrome.
ISSN:0008-5472
1538-7445