Ataxia-telangiectasia as a model system for studies of radiation protection mechanisms

Journal Article · Mon Aug 01 04:00:00 EDT 1988 · Health Phys.; (United States)

DOI:https://doi.org/10.1097/00004032-198808000-00023· OSTI ID:6729679

Painter, R B

Patients with ataxia-telangiectasia (AT), a human autosomal recessive genetic disease, are uniformly hypersensitive to ionizing radiation as measured by colony-forming ability and by chromosomal aberrations. Obligate heterozygotes, i.e., parents of AT patients, are slightly more radiosensitive than normal humans in terms of both colony-forming ability and chromosomal aberrations formed in G2. Thus, this system not only furnishes a model system to study factors that are responsible for radioresistance in normal human beings, but is also a unique tool for determining the role of gene dosage on radiation-induced cell killing. Because AT cells seem to be hypomutable to ionizing radiation, they also can be used to study the relationship between radiosensitivity and mutability and, therefore, carcinogenesis. Isolation of the defective gene that causes hypersensitivity in AT cells and its counterpart in normal cells should lead to a breakthrough in our understanding of radiation effects and how they can be prevented in human beings.

• Research Organization: Univ. of California, San Francisco (USA)
• OSTI ID: 6729679
• Journal Information: Health Phys.; (United States), Journal Name: Health Phys.; (United States) Vol. 55:2; ISSN HLTPA
• Country of Publication: United States
• Language: English

Related Subjects

560151* -- Radiation Effects on Animals-- Man
63 RADIATION, THERMAL, AND OTHER ENVIRON. POLLUTANT EFFECTS ON LIVING ORGS. AND BIOL. MAT.
CHROMOSOMAL ABERRATIONS
COLONY FORMATION
DISEASES
DNA
DOSES
MUTATIONS
NUCLEIC ACIDS
ORGANIC COMPOUNDS
PATIENTS
RADIATION DOSES
RADIATION PROTECTION
RADIOINDUCTION
RADIOSENSITIVITY
SKIN DISEASES
TELANGIECTASIS
VASCULAR DISEASES