Research Topics
Genomes and Genes | G WeedaSummaryAffiliation: Erasmus University Country: The Netherlands Publications
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Publications
The XPB subunit of repair/transcription factor TFIIH directly interacts with SUG1, a subunit of the 26S proteasome and putative transcription factorG Weeda
Department of Cell Biology, Medical Genetics Center, Erasmus University, Rotterdam, PO Box 1738, 3000 DR Rotterdam, The Netherlands
Nucleic Acids Res 25:2274-83. 1997..Since SUG1 is an integral component of the 26S proteasome and may be part of the mediator, our findings disclose a SUG1-dependent link between TFIIH and the cellular machinery involved in protein modelling/degradation...
A mutation in the XPB/ERCC3 DNA repair transcription gene, associated with trichothiodystrophyG Weeda
MGC Department of Cell Biology and Genetics, Erasmus University, Rotterdam, The Netherlands
Am J Hum Genet 60:320-9. 1997..quot;..
Mouse model for the DNA repair/basal transcription disorder trichothiodystrophy reveals cancer predispositionJ de Boer
MGC Department of Cell Biology and Genetics, Erasmus University, Rotterdam, The Netherlands
Cancer Res 59:3489-94. 1999..These findings have important implications for the etiology of the human disorder and for the impact of NER on carcinogenesis...
A mouse model for the basal transcription/DNA repair syndrome trichothiodystrophyJ de Boer
MGC Department of Cell Biology and Genetics Erasmus University, Rotterdam, The Netherlands
Mol Cell 1:981-90. 1998..The cutaneous symptoms are associated with reduced transcription of a skin-specific gene strongly supporting the concept of TTD as a human disease due to inborn defects in basal transcription and DNA repair...
Cloning of a human homolog of the yeast nucleotide excision repair gene MMS19 and interaction with transcription repair factor TFIIH via the XPB and XPD helicasesT Seroz
MGC Department of Cell Biology and Genetics, Center for Biomedical Genetics, Erasmus University Rotterdam, PO Box 1738, 3000 DR Rotterdam, The Netherlands
Nucleic Acids Res 28:4506-13. 2000..These findings extend the conservation of the NER apparatus and the link between NER and basal transcription and suggest that hMMS19 exerts its function in repair and transcription by interacting with the XPB and XPD helicases...
The structure-specific endonuclease Ercc1-Xpf is required for targeted gene replacement in embryonic stem cellsL J Niedernhofer
Department of Cell Biology and Genetics, Erasmus University Rotterdam, PO Box 1738, 3000 DR Rotterdam, The Netherlands
EMBO J 20:6540-9. 2001..We propose a model for the mechanism of targeted gene replacement that invokes a role for Ercc1-Xpf in making the recipient genomic locus receptive for gene replacement...
Disruption of the mouse xeroderma pigmentosum group D DNA repair/basal transcription gene results in preimplantation lethalityJ de Boer
Medical Genetics Center, Department of Cell Biology and Genetics, Erasmus University, Rotterdam, The Netherlands
Cancer Res 58:89-94. 1998....
Clinical heterogeneity within xeroderma pigmentosum associated with mutations in the DNA repair and transcription gene ERCC3W Vermeulen
MGC Department of Cell Biology and Genetics, Erasmus University, Rotterdam, The Netherlands
Am J Hum Genet 54:191-200. 1994..Factors in addition to NER deficiency may be required for the development of cutaneous tumors...
Correction of xeroderma pigmentosum repair defect by basal transcription factor BTF2 (TFIIH)A J van Vuuren
Department of Cell Biology and Genetics, Erasmus University, The Netherlands
EMBO J 13:1645-53. 1994..The remarkable dual role of ERCC3 in NER and transcription provides a clue in understanding the complex clinical features of some inherited repair syndromes...
Disruption of mouse ERCC1 results in a novel repair syndrome with growth failure, nuclear abnormalities and senescenceG Weeda
Department of Cell Biology and Genetics, Medical Genetics Center, Erasmus University, Rotterdam P O Box 1738, 3000 DR, Rotterdam, The Netherlands
Curr Biol 7:427-39. 1997....
Defective transcription-coupled repair in Cockayne syndrome B mice is associated with skin cancer predispositionG T van der Horst
Medical Genetics Center, Department of Cell Biology and Genetics, Erasmus University Rotterdam, The Netherlands
Cell 89:425-35. 1997..Further, they suggest that the lack of cancer predisposition in CS patients is attributable to a global genome repair process that in humans is more effective than in rodents...
Cloning and characterization of the Drosophila homolog of the xeroderma pigmentosum complementation-group B correcting gene, ERCC3M H Koken
Department of Cell Biology and Genetics, Erasmus University, Rotterdam, The Netherlands
Nucleic Acids Res 20:5541-8. 1992..g. B) fibroblasts and group 3 rodent mutants did not yield detectable correction. One of the possibilities to explain these negative findings is that the D.melanogaster protein may be unable to function in a mammalian repair context...
A new nucleotide-excision-repair gene associated with the disorder trichothiodystrophyM Stefanini
Consiglio Nazionale delle Richerche, Instituto di Genetica Biochemica Evoluzionistica, Pavia, Italy
Am J Hum Genet 53:817-21. 1993..The finding of a second DNA repair gene that is associated with the clinical features of TTD argues strongly for an involvement of repair proteins in hair-shaft development...
Molecular and functional analysis of the XPBC/ERCC-3 promoter: transcription activity is dependent on the integrity of an Sp1-binding siteL Ma
Sylvius Laboratories, University of Leiden, The Netherlands
Nucleic Acids Res 20:217-24. 1992..Band shift assays showed that this putative Sp1-binding site can interact specifically with a nuclear factor, most likely transcription factor Sp1 (or an Sp1-like factor) in vitro...
Transcription-coupled and global genome repair differentially influence UV-B-induced acute skin effects and systemic immunosuppressionJ Garssen
Laboratory for Pathology and Immunobiology and Laboratory of Health Effects Research, National Institute of Public Health and the Environment, Bilthoven, The Netherlands
J Immunol 164:6199-205. 2000....
The role of nucleotide excision repair in protecting embryonic stem cells from genotoxic effects of UV-induced DNA damageP P Van Sloun
Department of Radiation Genetics and Chemical Mutagenesis MGC, Leiden University Medical Center, PO Box 9503, 2300 RA Leiden, The Netherlands
Nucleic Acids Res 27:3276-82. 1999..Possibly, to avoid the accumulation of mutated cells, ES cells rely on the induction of a strong apoptotic response with a simultaneous shutting down of NER activity...
Characterization of the mouse homolog of the XPBC/ERCC-3 gene implicated in xeroderma pigmentosum and Cockayne's syndromeG Weeda
Laboratory for Molecular Carcinogenesis, Sylvius Laboratory, Leiden, The Netherlands
Carcinogenesis 12:2361-8. 1991..The mouse XPBC/ERCC-3 gene is expressed constitutively at low levels in all tissues examined except for testis, where its expression is significantly enhanced...
Localization of the xeroderma pigmentosum group B-correcting gene ERCC3 to human chromosome 2q21G Weeda
Laboratory for Molecular Carcinogenesis, Sylvius Laboratory, Leiden, The Netherlands
Genomics 10:1035-40. 1991..Cell Genet. 13: 539-551). Here we report its subchromosomal localization in the q21 region of chromosome 2 via somatic cell hybrids containing a translocated chromosome 2 and in situ hybridization with fluorescently labeled ERCC3 probes...
Cloning and characterization of p52, the fifth subunit of the core of the transcription/DNA repair factor TFIIHJ C Marinoni
Institut de Genetique et de Biologie Moleculaire et Cellulaire, Universite Louis Pasteur, Strasbourg, France
EMBO J 16:1093-102. 1997....
Structure and expression of the human XPBC/ERCC-3 gene involved in DNA repair disorders xeroderma pigmentosum and Cockayne's syndromeG Weeda
Laboratory for Molecular Carcinogenesis, Sylvius Laboratory, Leiden, The Netherlands
Nucleic Acids Res 19:6301-8. 1991..Southern blot analysis revealed the presence of XPBC/ERCC-3 cross-hybridizing fragments elsewhere in the genome, which may belong to a related gene...
Molecular cloning and biological characterization of the human excision repair gene ERCC-3G Weeda
Laboratory for Molecular Carcinogenesis, Sylvius Laboratory, Leiden, The Netherlands
Mol Cell Biol 10:2570-81. 1990..H. Thompson, A. V. Carrano, K. Sato, E. P. Salazar, B. F. White, S. A. Stewart, J. L. Minkler, and M. J. Siciliano, Somat. Cell. Mol. Genet. 13:539-551, 1987)...
A presumed DNA helicase encoded by ERCC-3 is involved in the human repair disorders xeroderma pigmentosum and Cockayne's syndromeG Weeda
Laboratory for Molecular Carcinogenesis, Sylvius Laboratory, Leiden, The Netherlands
Cell 62:777-91. 1990..Because XP is associated with predisposition to skin cancer, ERCC-3 can be considered a tumor-preventing gene...
The DNA-dependent ATPase activity associated with the class II basic transcription factor BTF2/TFIIHR Roy
UPR 6520 Centre National de la Recherche Scientifique, Unité 184 Institut National de la Santé et de la Recherche Médicale, Faculte de Medecine, Strasbourg, France
J Biol Chem 269:9826-32. 1994..Using recombinant wild type or mutated p89/ERCC3 polypeptides and different forms of DNA template, we show the connection between ATPase and the helicase...
The ERCC2/DNA repair protein is associated with the class II BTF2/TFIIH transcription factorL Schaeffer
UPR 6520 CNRS, Unité 184 INSERM, Faculte de Medecine, Strasbourg, France
EMBO J 13:2388-92. 1994..This includes clinical features that lack an adequate rationalization on the basis of nucleotide excision repair (NER) deficiency but which may now be explained better in terms of a partial transcription deficiency...
Base excision repair deficient mice lacking the Aag alkyladenine DNA glycosylaseB P Engelward
Department of Molecular and Cellular Toxicology, Harvard School of Public Health, Boston, MA 02115, USA
Proc Natl Acad Sci U S A 94:13087-92. 1997..Fibroblasts derived from Aag -/- mice are alkylation sensitive, indicating that Aag -/- mice may be similarly sensitive...
Hypermutation of immunoglobulin genes in memory B cells of DNA repair-deficient miceH Jacobs
Basel Institute for Immunology, CH 4005 Basel, Switzerland
J Exp Med 187:1735-43. 1998..This appears also to be true for mismatch repair, RAD54-dependent double-strand-break repair, and AAG-mediated base excision repair...
