Evidence for a repair enzyme complex involving ERCC1 and complementing activities of ERCC4, ERCC11 and xeroderma pigmentosum group F.

  title={Evidence for a repair enzyme complex involving ERCC1 and complementing activities of ERCC4, ERCC11 and xeroderma pigmentosum group F.},
  author={A. J. Vuuren and Esther Appeldoorn and H. Odijk and A. Yasui and N. Jaspers and D. Bootsma and J. Hoeijmakers},
  journal={The EMBO Journal},
Nucleotide excision repair (NER), one of the major cellular DNA repair systems, removes a wide range of lesions in a multi‐enzyme reaction. In man, a NER defect due to a mutation in one of at least 11 distinct genes, can give rise to the inherited repair disorders xeroderma pigmentosum (XP), Cockayne's syndrome or PIBIDS, a photosensitive form of the brittle hair disease trichothiodystrophy. Laboratory‐induced NER‐deficient mutants of cultured rodent cells have been classified into 11… Expand
ERCC4 (XPF) encodes a human nucleotide excision repair protein with eukaryotic recombination homologs
The ERCC4 gene structure and functional cDNA sequence encoding a 916-amino-acid protein has substantial homology with the eukaryotic DNA repair and recombination proteins MEI-9, Rad16, and Rad1 (Saccharomyces cerevisiae). Expand
Mechanism of cell cycle arrest in the ERCC1-deficient mouse model
The aim of this project was to gain a better understanding of the precise mechanisms behind the ERCC1 k/o phenotype, and it was concluded that the most likely explanation for the aneuploidsy and polyploidy the authors see in these livers is that these cells are arrested in G2. Expand
Partial characterization of the DNA repair protein complex, containing the ERCC1, ERCC4, ERCC11 and XPF correcting activities.
The partial purification and characterization of the ERCC1 complex is reported, finding the entire complex is considerably larger than previously found using size separation on glycerol gradients and has affinity for DNA, but no clear preference for ss, ds or UV-damaged DNA substrates. Expand
XPC and human homologs of RAD23: intracellular localization and relationship to other nucleotide excision repair complexes
Using heparin chromatography, gel filtration and native gel electrophoresis, it is demonstrated that the majority of HHR23B is in a free, non-complexed form, and that a minor fraction is tightly associated with XPC, while the H HR23A may have an additional function independent of XPC. Expand
Xeroderma Pigmentosum Group F Caused by a Defect in a Structure-Specific DNA Repair Endonuclease
Results demonstrate that the XPF, ERCC4, and ERCC11 genes are equivalent, complete the isolation of the XP genes that form the core nucleotide excision repair system, and solve the catalytic function of theXPF-containing complex. Expand
The XPA-binding domain of ERCC1 Is Required for Nucleotide Excision Repair but Not Other DNA Repair Pathways*
The studies suggest that ERCC1-XPF engages in different repair pathways through specific protein-protein interactions and that these functions can be separated through the selective disruption of these interactions. Expand
A 3′ → 5′ XPB Helicase Defect in Repair/Transcription Factor TFIIH of Xeroderma Pigmentosum Group B Affects Both DNA Repair and Transcription*
  • Jae Ryoung Hwang, V. Moncollin, +4 authors J. Egly
  • Biology, Medicine
  • The Journal of Biological Chemistry
  • 1996
This work presents the first detailed analysis of a naturally occurring mutation in a basal transcription factor and supports the concept that the combined XP/CS clinical entity is actually the result of a combined transcription/repair deficiency. Expand
Activity of individual ERCC1 and XPF subunits in DNA nucleotide excision repair.
Protein sequence comparison revealed similarity between the ERCC1 family and the C-terminal region of the XPF family, including the regions of both proteins that are necessary for the ER CC1-XPF heterodimeric interaction, which suggests that the ERcc1 and XPF families are related via an ancient duplication. Expand
XPF knockout via CRISPR/Cas9 reveals that ERCC1 is retained in the cytoplasm without its heterodimer partner XPF
It is shown that human XPF is markedly involved in homologous recombination repair (HRR) but dispensable for non-homologous end-joining (NHEJ), and Notably, ERCC1 was not detectable in the nucleus of the XPF knockout cells indicating the necessity of a functional XPF/ERCC1 heterodimer to allow ER CC1 to enter the nucleus. Expand
The importance of the ERCC1/ERCC4[XPF] complex for hypoxic-cell radioresistance does not appear to derive from its participation in the nucleotide excision repair pathway.
It is concluded that the increased hypoxic radiosensitivity observed in the UV20 and UV41 mutants is due to a defect in the ERCC1/ERCC4-dependent pathway for the repair of DNA cross-links and not in the nucleotide excision repair pathway. Expand


Stable and specific association between the yeast recombination and DNA repair proteins RAD1 and RAD10 in vitro.
The results are the first biochemical evidence of a specific association between any eukaryotic proteins genetically identified as belonging to a recombination or DNA repair pathway and suggest that the RAD1 and RAD10 proteins act at the same or consecutive biochemical steps in both nucleotide excision repair and mitotic recombination. Expand
An eighth complementation group of rodent cells hypersensitive to ultraviolet radiation
The mouse mutant US31 produced UV-resistant hybrid cells in each of the seven crosses, indicating that it forms an eighth complementation group among the rodent mutants, suggesting that at least eight genes are likely required to repair UV damage in rodent cells. Expand
Xeroderma pigmentosum. The Metabolic Basisfor Inherited Disease
  • 1989