Nucleic Acids Research, Vol 24, Issue 17 3370-3380, Copyright © 1996 by Oxford University Press
AM Sijbers, PJ van der Spek, H Odijk, J van den Berg, M van Duin, A Westerveld, NG Jaspers, D Bootsma and JH Hoeijmakers
The human DNA repair protein ERCC1 resides in a complex together with the
ERCC4, ERCC11 and XP-F correcting activities, thought to perform the 5'
strand incision during nucleotide excision repair (NER). Its yeast
counterpart, RAD1-RAD10, has an additional engagement in a mitotic
recombination pathway, probably required for repair of DNA cross-links.
Mutational analysis revealed that the poorly conserved N- terminal 91 amino
acids of ERCC1 are dispensable for both repair functions, in contrast to a
deletion of only four residues from the C- terminus. A database search
revealed a strongly conserved motif in this C-terminus sharing sequence
homology with many DNA break processing proteins, indicating that this part
is primarily required for the presumed structure-specific endonuclease
activity of ERCC1. Most missense mutations in the central region give rise
to an unstable protein (complex). Accordingly, we found that free ERCC1 is
very rapidly degraded, suggesting that protein-protein interactions provide
stability. Survival experiments show that the removal of cross-links
requires less ERCC1 than UV repair. This suggests that the ERCC1- dependent
step in cross-link repair occurs outside the context of NER and provides an
explanation for the phenotype of the human repair syndrome xeroderma
pigmentosum group F.
ARTICLES
Mutational analysis of the human nucleotide excision repair gene ERCC1
Department of Cell Biology and Genetics, Erasmus University, Rotterdam, The Netherlands.
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