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Adv Exp Med Biol. 2008;637:65-82.

XPF/ERCC4 and ERCC1: their products and biological roles.

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  • 1Department of Pathology, University of Texas Southwestern Medical Center at Dallas, Dallas, Texas 75390-9072, USA.


ERCC4 is the gene mutated in XPF cells and also in rodent cells representing the mutant complementation groups ERCC4 and ERCC 11. The protein functions principally as a complex with ERCC1 in a diversity of biological pathways that include NER, ICL repair, telomere maintenance and immunoglobulin switching. Sorting out these roles is an exciting and challenging problem and many important questions remain to be answered. The ERCC1/ERCC4 complex is conserved across most species presenting an opportunity to examine some functions in model organisms where mutants can be more readily generated and phenotypes more quickly assessed.

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