Fanconi anaemia and the repair of Watson and Crick DNA crosslinks
Top Cited Papers
- 16 January 2013
- journal article
- review article
- Published by Springer Science and Business Media LLC in Nature
- Vol. 493 (7432), 356-363
- https://doi.org/10.1038/nature11863
Abstract
The function of Fanconi anaemia proteins is to maintain genomic stability. Their main role is in the repair of DNA interstrand crosslinks, which, by covalently binding the Watson and the Crick strands of DNA, impede replication and transcription. Inappropriate repair of interstrand crosslinks causes genomic instability, leading to cancer; conversely, the toxicity of crosslinking agents makes them a powerful chemotherapeutic. Fanconi anaemia proteins can promote stem-cell function, prevent tumorigenesis, stabilize replication forks and inhibit inaccurate repair. Recent advances have identified endogenous aldehydes as possible culprits of DNA damage that may induce the phenotypes seen in patients with Fanconi anaemia.Keywords
This publication has 75 references indexed in Scilit:
- Mutations in BRIP1 confer high risk of ovarian cancerNature Genetics, 2011
- Human SNM1A and XPF–ERCC1 collaborate to initiate DNA interstrand cross-link repairGenes & Development, 2011
- Functional and physical interaction between the mismatch repair and FA-BRCA pathwaysHuman Molecular Genetics, 2011
- How high are carrier frequencies of rare recessive syndromes? Contemporary estimates for Fanconi Anemia in the United States and IsraelAmerican Journal of Medical Genetics Part A, 2011
- Fancd2 counteracts the toxic effects of naturally produced aldehydes in miceNature, 2011
- Mechanism of RAD51-Dependent DNA Interstrand Cross-Link RepairScience, 2011
- DNA interstrand crosslink repair and cancerNature Reviews Cancer, 2011
- Double-Strand Break Repair-Independent Role for BRCA2 in Blocking Stalled Replication Fork Degradation by MRE11Cell, 2011
- Aberrant chromosome morphology in human cells defective for Holliday junction resolutionNature, 2011
- SLX4, a coordinator of structure-specific endonucleases, is mutated in a new Fanconi anemia subtypeNature Genetics, 2011