Article
RPA-Mediated Recruitment of the E3 Ligase RFWD3 Is Vital for Interstrand Crosslink Repair and Human Health.
Molecular cell - 1 Jun 2017
Feeney Laura, Muñoz Ivan M, Lachaud Christophe, Toth Rachel, Appleton Paul L, Schindler Detlev, Rouse John
Abstract excerpt
Defects in the repair of DNA interstrand crosslinks (ICLs) are associated with the genome instability syndrome Fanconi anemia (FA). Here we report that cells with mutations in RFWD3, an E3 ubiquitin ligase that interacts with and ubiquitylates replication protein A (RPA), show profound defects in ICL repair. An amino acid substitution in the WD40 repeats of RFWD3 (I639K) found in a new FA subtype abolishes...
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