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Enhanced radiosensitivity and defective DNA repair in cultured fibroblasts derived from Rothmund Thomson syndrome patients

Journal Article:

Abstract

Rothmund Thomson syndrome (RTS) is an oculocutaneous and cancer-prone disorder in which enhanced carcinogen sensitivity, mediated through abnormal DNA metabolism, may be an associated factor. Cultured fibroblasts from 4 RTS patients have been examined for their colony-forming abilities and DNA repair capacities following ..gamma..-irradiation. 2 of the 4 RTS strains showed enhanced sensitivity following hypoxic ..gamma..-irradiation, and 1 of these 2 strains also showed enhanced sensitivity under oxic conditions. Defective DNA repair was implicated in the above abnormal responses to ..gamma..-radiation since both strains displayed reduced levels of repair synthesis and slow removal of radiogenic DNA lesions (assayed by their sensitivity to strand-incising activities present in protein extracts of Micrococcus luteus cells). A hypothesis is presented to rationalize the origin and heterogeneity of these laboratory phenotypes of RTS.
Authors:
Smith, P J; Paterson, M C [1] 
  1. Atomic Energy of Canada Ltd., Chalk River, Ontario. Radiation Biology Branch
Publication Date:
Jan 01, 1982
Product Type:
Journal Article
Reference Number:
AIX-13-699051; EDB-82-178872
Resource Relation:
Journal Name: Mutat. Res.; (Netherlands); Journal Volume: 94
Subject:
63 RADIATION, THERMAL, AND OTHER ENVIRON. POLLUTANT EFFECTS ON LIVING ORGS. AND BIOL. MAT.; FIBROBLASTS; RADIOSENSITIVITY; ANIMAL CELLS; BIOLOGICAL REPAIR; CELL CULTURES; DNA; GAMMA RADIATION; STRAND BREAKS; SURVIVAL CURVES; BIOLOGICAL RECOVERY; CONNECTIVE TISSUE CELLS; ELECTROMAGNETIC RADIATION; IONIZING RADIATIONS; NUCLEIC ACIDS; ORGANIC COMPOUNDS; RADIATIONS; RECOVERY; REPAIR; SOMATIC CELLS; 560121* - Radiation Effects on Cells- External Source- (-1987)
OSTI ID:
6793100
Country of Origin:
Netherlands
Language:
English
Other Identifying Numbers:
Journal ID: CODEN: MUREA
Submitting Site:
HEDB
Size:
Pages: 213-228
Announcement Date:

Journal Article:

Citation Formats

Smith, P J, and Paterson, M C. Enhanced radiosensitivity and defective DNA repair in cultured fibroblasts derived from Rothmund Thomson syndrome patients. Netherlands: N. p., 1982. Web.
Smith, P J, & Paterson, M C. Enhanced radiosensitivity and defective DNA repair in cultured fibroblasts derived from Rothmund Thomson syndrome patients. Netherlands.
Smith, P J, and Paterson, M C. 1982. "Enhanced radiosensitivity and defective DNA repair in cultured fibroblasts derived from Rothmund Thomson syndrome patients." Netherlands.
@misc{etde_6793100,
title = {Enhanced radiosensitivity and defective DNA repair in cultured fibroblasts derived from Rothmund Thomson syndrome patients}
author = {Smith, P J, and Paterson, M C}
abstractNote = {Rothmund Thomson syndrome (RTS) is an oculocutaneous and cancer-prone disorder in which enhanced carcinogen sensitivity, mediated through abnormal DNA metabolism, may be an associated factor. Cultured fibroblasts from 4 RTS patients have been examined for their colony-forming abilities and DNA repair capacities following ..gamma..-irradiation. 2 of the 4 RTS strains showed enhanced sensitivity following hypoxic ..gamma..-irradiation, and 1 of these 2 strains also showed enhanced sensitivity under oxic conditions. Defective DNA repair was implicated in the above abnormal responses to ..gamma..-radiation since both strains displayed reduced levels of repair synthesis and slow removal of radiogenic DNA lesions (assayed by their sensitivity to strand-incising activities present in protein extracts of Micrococcus luteus cells). A hypothesis is presented to rationalize the origin and heterogeneity of these laboratory phenotypes of RTS.}
journal = {Mutat. Res.; (Netherlands)}
volume = {94}
journal type = {AC}
place = {Netherlands}
year = {1982}
month = {Jan}
}