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.
Smith, P J;
Paterson, M C
[1]
- Atomic Energy of Canada Ltd., Chalk River, Ontario. Radiation Biology Branch
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 = []
volume = {94}
journal type = {AC}
place = {Netherlands}
year = {1982}
month = {Jan}
}
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 = []
volume = {94}
journal type = {AC}
place = {Netherlands}
year = {1982}
month = {Jan}
}