1979
DOI: 10.1002/jcp.1041010211
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Specific cellular defects in patients with Fanconi anemia

Abstract: Measurements of plating efficiency, accumulation of metaphases and generation times have shown that fibroblast from patients with Fanconi anemia (FA) have decreased probability of completing a further division after successful mitosis. Thus FA cells show decreased growth rates and increased generation times. We have also measured the survival of FA fibroblasts and lymphoblasts after treatment with a variety of mutagens. All FA cells show an increased sensitivity to drugs such as MMC and psoralen plus long wave… Show more

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Cited by 106 publications
(22 citation statements)
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“…1). TS fibroblasts thus probably do not bear resemblance to chromosomal instability syndromes such as AT, Fanconi anemia, or Bloom syndrome, with respect to the growth rate Swift, 1976a, 1976b;Weksberg et al, 1979) and to the colony-forming efficiency (Shiloh et al, 1982).…”
Section: Discussionmentioning
confidence: 99%
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“…1). TS fibroblasts thus probably do not bear resemblance to chromosomal instability syndromes such as AT, Fanconi anemia, or Bloom syndrome, with respect to the growth rate Swift, 1976a, 1976b;Weksberg et al, 1979) and to the colony-forming efficiency (Shiloh et al, 1982).…”
Section: Discussionmentioning
confidence: 99%
“…MNNG was dissolved in dimethylsulfoxide at a concentration of 10 mg/ml and kept at -20~ 4NQO was dissolved in ethanol at a concentration of 250 yg/ml and kept at -20~ These compounds were diluted into medium immediately before use. After cells were exposed to each chemical for 24 hours, the periods being determined according to Weksberg et al (1979), cells were rinsed three times with warmed medium without FBS. Then fresh DEM containing 10 FBS was added to and the cells were cultured.…”
Section: Methodsmentioning
confidence: 99%
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“…These patients show a high level of chromatid-type damage in their lymphocytes and fibroblasts (2)(3)(4). Cultured FA cells are unusually sensitive to DNA crosslinking agents by chromosomal criteria (5)(6)(7)(8)(9) and by clonogenic cell survival (10)(11)(12)(13)(14) whereas their sensitivity to monofunctional agents or to radiation is close to normal (12,15).…”
Section: Introductionmentioning
confidence: 99%
“…In particular, their G 2 phase is unusually long, and many cells remain permanently arrested within this phase (44,47,60). G 2 arrest may reflect the accumulation of DNA damage and the need to repair this damage before mitosis.…”
mentioning
confidence: 99%