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repair mutation ercc 1-xpf ercc 1 protein 1-xpf damage ercc 1à/à mice recombination lesion cancer excision patient csbm /m mice figure fancd xpf-ercc h 2ax foci nucleotide dna repair defect level syndrome mouse liver nucleotide excision repair mechanism endonuclease junction wild-type dna damage hematopoietic response growth pathway culture hoeijmaker ageing fi broblasts ercc 1ϫ/ϫ cells segment switch table littermate dsb repair xpf-ercc 1 sequence class fraction wt cells cross-link embo j region broblast signi result ficient b cells fancd 2 antibody progenitors icl repair csbm /m wild-type cells signi ficantly cause number wt littermates expression oxidative xpfr 153p phenotype wt mice genetic activity formation anemia double-strand 165 tor cells progenitor
10 Most Recent Publications
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Mislocalization of XPF-ERCC1 nuclease contributes to reduced DNA repair in XP-F patients
(Article)
Ahmad, R.A. Enzlin, J.H. Schärer, O.D. Niedernhofer, L.J. Bhagwat, N. Wijgers, N. Raams, A. Appledoorn, E. Theil, A.F. Hoeijmakers, J.H.J. Vermeulen, W. Jaspers, N.G.J. |
2010-03-01
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XPF-ERCC1 participates in the Fanconi anemia pathway of cross-link repair
(Article)
Bhagwat, N. Olsen, A.L. Wang, A.T. Hanada, K. Stuckert, P. Kanaar, R. D'Andrea, A. Niedernhofer, L.J. McHugh, P.J. |
2009-12-01
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Delayed and accelerated aging share common longevity assurance mechanisms
(Article)
Schumacher, B. Pluijm, I. van der Bartke, A. Spindler, S.R. Hoeijmakers, J.H.J. Horst, G.T.J. van der Garinis, G.A. Moorhouse, M.J. Kosteas, T. Robinson, A.R. Suh, Y. Breit, T.M. Steeg, H. van Niedernhofer, L.J. IJcken, W.F.J. van |
2008-08-01
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ERCC1-XPF endonuclease facilitates DNA double-strand break repair
(Article)
Ahmad, R.A. Robinson, A.R. Duensing, A. Drunen, E. van Beverloo, H.B. Weisberg, D.B. Hasty, P. Hoeijmakers, J.H.J. Niedernhofer, L.J. |
2008-08-01
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First reported patient with human ERCC1 deficiency has cerebro-oculo-facio- skeletal syndrome with a mild defect in nucleotide excision repair and severe developmental failure
(Article)
Jaspers, N.G.J. Raams, A. Vermeulen, W. Silengo, M.C. Wijgers, N. Niedernhofer, L.J. Robinson, A.R. Giglia-Mari, G. Hoogstraten, D. Kleijer, W.J. Hoeijmakers, J.H.J. |
2007-03-01
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Impaired genome maintenance suppresses the growth hormone--insulin-like growth factor 1 axis in mice with Cockayne syndrome.
(Article)
Pluijm, I. van der Garinis, G.A. Niedernhofer, L.J. Velasco, S. Friedberg, E.C. Tanaka, K. Steeg, H. van Hoeijmakers, J.H.J. Horst, G.T.J. van der Brandt, R.M.C. Gorgels, T.G.M.F. Wijnhoven, S.W.P. Diderich, K. Wit, J. de Mitchell, J.R. Oostrom, C.T.M. van Beems, R.B. |
2006-12-12
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Reduced hematopoietic reserves in DNA interstrand crosslink repair- deficient Ercc1-/- mice.
(Article)
Prasher, J.M. Lalai, A.S. Heijmans-Antonissen, C. Ploemacher, R.E. Hoeijmakers, J.H.J. Touw, I.P. Niedernhofer, L.J. |
2005-02-23
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Deletion of the nucleotide excision repair gene Ercc1 reduces immunoglobulin class switching and alters mutations near switch recombination junction
(Article)
Schrader, C.E. Vardo, J. Linehan, E. Twarog, M.Z. Niedernhofer, L.J. Hoeijmakers, J.H.J. Stavezner, J. |
2004-01-01
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Deletion of the nucleotide excision repair gene Ercc1 reduces immunoglobulin class switching and alters mutations near switch recombination junctions
(Article)
Schrader, C.E. Vardo, J. Linehan, E. Twarog, M.Z. Niedernhofer, L.J. Stavnezer, J. Hoeijmakers, J.H.J. |
2004-01-01
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The structure-specific endonuclease Ercc1-Xpf is required to resolve DNA insterstrand cross-link-induced double-strand breaks
(Article)
Niedernhofer, L.J. Odijk, H. Kanaar, R. Budzowska, M. Drunen, E. van Maas, A. Theil, A.F. Wit, J. de Beverloo, H.B. Hoeijmakers, J.H.J. Jaspers, N.G.J. |
2004-01-01
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