In vitro phenotype of ataxia-telangiectasia (AT) fibroblast strains: clues to the nature of the ''AT DNA lesion'' and the molecular defect in AT

Journal Article · Tue Jan 01 00:00:00 EST 1985 · KROC Found. Ser.; (United States)

OSTI ID:5738666

Shiloh, Y; Tabor, E; Becker, Y

Studies of the in vitro phenotype of a series of AT strains established in Israel revealed the following features: premature senescence and increased demands for growth factors, normal sensitivity to the cytotoxic effect of alkylating agents, hypersensitivity to agents that damage the deoxyribose moiety of DNA via a ''targeted'' free radical attack (this hypersensitivity is coupled with reduced inhibition of DNA synthesis compared to normal cells), varying degrees of intermediate hypersensitivity to the same agents in AT heterozygous cells, lack of potentially lethal damage repair and sublethal damage repair in AT homozygous cells following treatment with free radical-producing agents. We conclude that AT involves a DNA repair defect and that the AT DNA lesion is probably a gap with the 3'-phosphate or 3'-phosphoglycolate end left in the DNA following sugar destruction.

• OSTI ID: 5738666
• Journal Information: KROC Found. Ser.; (United States), Journal Name: KROC Found. Ser.; (United States)
• Country of Publication: United States
• Language: English

Related Subjects

63 RADIATION, THERMAL, AND OTHER ENVIRON. POLLUTANT EFFECTS ON LIVING ORGS. AND BIOL. MAT.
59 BASIC BIOLOGICAL SCIENCES
FIBROBLASTS
PHENOTYPE
RADIOSENSITIVITY
ALKYLATING AGENTS
BIOCHEMISTRY
CONGENITAL DISEASES
DNA REPAIR
DNA REPLICATION
PATHOGENESIS
PATIENTS
VASCULAR DISEASES
ANIMAL CELLS
BIOLOGICAL RECOVERY
BIOLOGICAL REPAIR
CHEMISTRY
CONNECTIVE TISSUE CELLS
DISEASES
NUCLEIC ACID REPLICATION
RECOVERY
REPAIR
SOMATIC CELLS
560121* - Radiation Effects on Cells- External Source- (-1987)
550900 - Pathology