Fanconi anaemia and the repair of Watson and Crick DNA crosslinks

Review

关键词: STRUCTURE-SPECIFIC ENDONUCLEASES;    HOLLIDAY JUNCTION RESOLVASE;    CANCER SUSCEPTIBILITY GENE;    BREAK REPAIR;    HOMOLOGOUS RECOMBINATION;    MONOUBIQUITINATED FANCD2;    HEMATOPOIETIC STEM;    CELLS DEFICIENT;    HELICASE BRIP1;    OVARIAN-CANCER;   

DOI  :  10.1038/nature11863

来源: SCIE

【 摘 要 】

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.

【 授权许可】

Free