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- W2015626135 endingPage "13748" @default.
- W2015626135 startingPage "13736" @default.
- W2015626135 abstract "Fanconi anaemia (FA) is a genome instability disease caused by defects in the FA DNA repair pathway that senses and repairs damage caused by DNA interstrand crosslinks. At least 8 of the 16 genes found mutated in FA encode proteins that assemble into the FA core complex, a multisubunit monoubiquitin E3 ligase. Here, we show that the RuvBL1 and RuvBL2 AAA+ ATPases co-purify with FA core complex isolated under stringent but native conditions from a vertebrate cell line. Depletion of the RuvBL1-RuvBL2 complex in human cells causes hallmark features of FA including DNA crosslinker sensitivity, chromosomal instability and defective FA pathway activation. Genetic knockout of RuvBL1 in a murine model is embryonic lethal while conditional inactivation in the haematopoietic stem cell pool confers profound aplastic anaemia. Together these findings reveal a function for RuvBL1-RuvBL2 in DNA repair through a physical and functional association with the FA core complex. Surprisingly, depletion of RuvBL1-RuvBL2 leads to co-depletion of the FA core complex in human cells. This suggests that a potential mechanism for the role of RuvBL1-RuvBL2 in maintaining genome integrity is through controlling the cellular abundance of FA core complex." @default.
- W2015626135 created "2016-06-24" @default.
- W2015626135 creator A5026689567 @default.
- W2015626135 creator A5034456496 @default.
- W2015626135 creator A5049321963 @default.
- W2015626135 creator A5060928128 @default.
- W2015626135 date "2014-11-26" @default.
- W2015626135 modified "2023-09-24" @default.
- W2015626135 title "Abundance of the Fanconi anaemia core complex is regulated by the RuvBL1 and RuvBL2 AAA+ ATPases" @default.
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- W2015626135 doi "https://doi.org/10.1093/nar/gku1230" @default.