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- W2027545181 abstract "Bloom syndrome (BS) and ataxia-telangiectasia (A-T) are rare autosomal recessive diseases associated with chromosomal instability. The genes responsible for BS and A-T have been identified as BLM and ATM, respectively, whose products were recently found to be components of BRCA1-associated genome surveillance complex (BASC), a supercomplex possibly involved in the recognition and repair of aberrant DNA structures. Based on experiments using BLM−/− DT40 cells and BLM−/−/RAD54−/− DT40 cells, we previously suggested that BLM functions to reduce the formation of double-strand breaks (DSBs) during DNA replication. To examine whether ATM is involved in the recognition and/or repair of DSBs generated in BLM−/− DT40 cells and to address the functional relationship between the two BASC components, we generated BLM−/−/ATM−/− DT40 cells and characterized their properties as well as those of ATM−/− and BLM−/− DT40 cells. BLM−/−/ATM−/− cells proliferated slightly more slowly than either BLM−/− or ATM−/− cells. The sensitivity of BLM−/−/ATM−/− cells to γ-irradiation was similar to that of ATM−/− cells, while BLM−/− cells were slightly resistant to γ-irradiation compared with wild-type cells. BLM−/− cells showed sensitivity to methyl methanesulfonate (MMS) and UV irradiation while ATM−/− cells did not show sensitivity to either agent. The sensitivity of BLM−/−/ATM−/− cells to MMS and UV was similar to that of BLM−/− cells. Disrupting the function of ATM reduced the targeted integration frequency in BLM−/− DT40 cells. However, a defect in ATM only slightly reduced the increased sister chromatid exchanges (SCEs) in BLM−/− DT40 cells." @default.
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- W2027545181 date "2004-03-01" @default.
- W2027545181 modified "2023-10-17" @default.
- W2027545181 title "The absence of a functional relationship between ATM and BLM, the components of BASC, in DT40 cells" @default.
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- W2027545181 doi "https://doi.org/10.1016/j.bbadis.2003.11.008" @default.
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