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- Title
Ubiquitylation and the Fanconi anemia pathway
- Authors
Garner, Elizabeth; Smogorzewska, Agata
- Abstract
Abstract: The Fanconi anemia (FA) pathway maintains genome stability through co-ordination of DNA repair of interstrand crosslinks (ICLs). Disruption of the FA pathway yields hypersensitivity to interstrand crosslinking agents, bone marrow failure and cancer predisposition. Early steps in DNA damage dependent activation of the pathway are governed by monoubiquitylation of FANCD2 and FANCI by the intrinsic FA E3 ubiquitin ligase, FANCL. Downstream FA pathway components and associated factors such as FAN1 and SLX4 exhibit ubiquitin-binding motifs that are important for their DNA repair function, underscoring the importance of ubiquitylation in FA pathway mediated repair. Importantly, ubiquitylation provides the foundations for cross-talk between repair pathways, which in concert with the FA pathway, resolve interstrand crosslink damage and maintain genomic stability.
- Subjects
FANCONI'S anemia; UBIQUITIN; GENOMES; DISEASE susceptibility; DNA repair; DNA damage; DNA ligases; ALLERGIES
- Publication
FEBS Letters, 2011, Vol 585, Issue 18, p2853
- ISSN
0014-5793
- Publication type
Article
- DOI
10.1016/j.febslet.2011.04.078