Heterogeneous Response to X-Ray and Ultraviolet Light Irradiations of Cultured Skin Fibroblasts in Two Families With Gardner's Syndrome

A heterogeneous response to X-ray and far UV (254 nm) light irradiations was found in cultured skin fibroblast lines from 2 separate families with Gardner's syndrome. When compared to 2 normal control cultures and cultures from 2 patients with nonfamilial colon cancer, cultures from 4 clinicall...

Full description

Saved in:
Bibliographic Details
Published in:JNCI : Journal of the National Cancer Institute 1982-05, Vol.68 (5), p.697-701
Main Authors: Kinsella, Timothy J., Little, John B., Nove, John, Weichselbaum, Ralph R., Li, Frederick P., Meyer, Robert J., Marchetto, David J., Patterson, W. Bradford
Format: Article
Language:English
Subjects:
Adolescent
Adult
Cell Survival
Cells, Cultured
DNA Repair
Gardner Syndrome - genetics
Gardner Syndrome - pathology
Humans
Infant, Newborn
Karyotyping
Middle Aged
Phenotype
Radiation Tolerance
Risk
Skin - pathology
Skin - radiation effects
Ultraviolet Rays
X-Rays
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:A heterogeneous response to X-ray and far UV (254 nm) light irradiations was found in cultured skin fibroblast lines from 2 separate families with Gardner's syndrome. When compared to 2 normal control cultures and cultures from 2 patients with nonfamilial colon cancer, cultures from 4 clinically affected members of family 1 showed increased sensitivity to the lethal effects of both X-ray and UV light irradiations. These cell lines also showed a delayed pattern. of Xray potentially lethal damage repair (PLDR) and absent UV PLDR. In contrast, cultures from 3 members of family 2 (2 of whom were clinically affected) showed a normal response of survival and PLDR to both Xray and UV light irradiations. Thus increased sensitivity of cultured skin fibroblasts to X-ray and UV light irradiations was not a consistent in vitro finding in patients with Gardner's syndrome. However, in families with Gardner's syndrome who demonstrate in vitro radiosensitivity, additional studies are needed to assess the usefulness of these techniques in detecting affected individuals prior to the development of colon carcinoma and other manifestations.
ISSN:0027-8874
1460-2105
DOI:10.1093/jnci/68.5.697