LAURA JANE NIEDERNHOFER
Affiliation: University of Pittsburgh
- Mislocalization of XPF-ERCC1 nuclease contributes to reduced DNA repair in XP-F patientsAnwaar Ahmad
Department of Microbiology and Molecular Genetics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States of America
PLoS Genet 6:e1000871. 2010..Analysis of these patient cells therefore reveals a novel mechanism to potentially regulate a cell's capacity for DNA repair: by manipulating nuclear localization of XPF-ERCC1...
- Signaling mechanisms involved in the response to genotoxic stress and regulating lifespanLaura J Niedernhofer
Department of Molecular Genetics and Biochemistry, University of Pittsburgh School of Medicine, University of Pittsburgh Cancer Institute, PA 15213, USA
Int J Biochem Cell Biol 40:176-80. 2008..This review highlights the significant overlap in signaling mechanisms implicated in both the cellular response to genotoxic stress and regulation of organism lifespan...
- DNA repair is crucial for maintaining hematopoietic stem cell functionLaura J Niedernhofer
Department of Molecular Genetics and Biochemistry, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA
DNA Repair (Amst) 7:523-9. 2008..These studies strongly support the hypothesis that genomic stress causes aging by limiting the ability of stem cells to indefinitely maintain tissue homeostasis...
- The Fanconi anemia signalosome anchorLaura J Niedernhofer
Department of Molecular Genetics and Biochemistry, University of Pittsburgh School of Medicine and Cancer Institute, Pittsburgh, PA 15213, USA
Mol Cell 25:487-90. 2007....
- Nucleotide excision repair deficient mouse models and neurological diseaseLaura J Niedernhofer
Department of Microbiology and Molecular Genetics, University of Pittsburgh School of Medicine, Hillman Cancer Center, Pittsburgh, PA 15213, USA
DNA Repair (Amst) 7:1180-9. 2008..This review explores the utility of these mouse models to study neurological disease caused by NER-deficiency...
- Tissue-specific accelerated aging in nucleotide excision repair deficiencyLaura J Niedernhofer
Department of Microbiology and Molecular Genetics, UP Cancer Institute, University of Pittsburgh School of Medicine, 5117 Centre Avenue, Pittsburgh, PA 15213, USA
Mech Ageing Dev 129:408-15. 2008..This review emphasizes accelerated aging associated with xeroderma pigmentosum and discusses the cause of these pathologies, either mutation accumulation or cell death as a consequence of failure to repair DNA damage...
- The structure-specific endonuclease Ercc1-Xpf is required to resolve DNA interstrand cross-link-induced double-strand breaksLaura J Niedernhofer
Department of Cell Biology and Genetics, Erasmus Medical Center, Rotterdam, The Netherlands
Mol Cell Biol 24:5776-87. 2004..Collectively, these data support a role for Ercc1-Xpf in processing ICL-induced DSBs so that these cytotoxic intermediates can be repaired by homologous recombination...
- ERCC1-XPF endonuclease facilitates DNA double-strand break repairAnwaar Ahmad
University of Pittsburgh Cancer Institute, Hillman Cancer Center, Research Pavilion 2 6, 5117 Centre Avenue, Pittsburgh, Pennsylvania 15213 1863, USA
Mol Cell Biol 28:5082-92. 2008..These data support the conclusion that, as in yeast, ERCC1-XPF facilitates DSB repair via an end-joining mechanism that is Ku86 independent...
- A new progeroid syndrome reveals that genotoxic stress suppresses the somatotroph axisLaura J Niedernhofer
Center for Biomedical Genetics Medical Genetic Center Department of Cell Biology and Genetics, Erasmus Medical Center, PO Box 1738, 3000 DR Rotterdam, The Netherlands
Nature 444:1038-43. 2006....
- Reduced hematopoietic reserves in DNA interstrand crosslink repair-deficient Ercc1-/- miceJoanna M Prasher
Department of Hematology, Erasmus Medical Center, Rotterdam, The Netherlands
EMBO J 24:861-71. 2005..These data support the hypothesis that spontaneous interstrand crosslink damage contributes to the functional decline of the hematopoietic system associated with aging...
- Impaired genome maintenance suppresses the growth hormone--insulin-like growth factor 1 axis in mice with Cockayne syndromeIngrid van der Pluijm
Department of Genetics, Center for Biomedical Genetics, Erasmus University Medical Center, Rotterdam, The Netherlands
PLoS Biol 5:e2. 2007..Importantly, wild-type mice exposed to a low dose of chronic genotoxic stress recapitulated this response, thereby pointing to a novel link between genome instability and the age-related decline of the somatotroph axis...
- XPF-ERCC1 participates in the Fanconi anemia pathway of cross-link repairNikhil Bhagwat
Department of Human Genetics, University of Pittsburgh School of Public Health, A300 Crabtree Hall, 130 DeSoto St, Pittsburgh, Pennsylvania 152611, USA
Mol Cell Biol 29:6427-37. 2009..These data demonstrate that the unhooking of an ICL by XPF-ERCC1 is necessary for the stable localization of FANCD2 to the chromatin and subsequent homologous recombination-mediated DSB repair...
- Accelerated aging of intervertebral discs in a mouse model of progeriaNam Vo
Ferguson Laboratory for Orthopaedic Research, Department of Orthopaedic Surgery, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania 15261, USA
J Orthop Res 28:1600-7. 2010..These results demonstrate that Ercc1(-/Δ) mice represent an accurate and rapid model of disc aging and provide novel evidence that DNA damage negatively impacts PG synthesis...
- First reported patient with human ERCC1 deficiency has cerebro-oculo-facio-skeletal syndrome with a mild defect in nucleotide excision repair and severe developmental failureNicolaas G J Jaspers
Department of Genetics, Erasmus Medical Center, Rotterdam, The Netherlands
Am J Hum Genet 80:457-66. 2007..This discovery represents a novel complementation group of patients with defective NER. Further, the clinical severity, coupled with a relatively mild repair defect, suggests novel functions for ERCC1...
- Divide and conquer: nucleotide excision repair battles cancer and ageingJames R Mitchell
Department of Cell Biology and Genetics, Erasmus University Rotterdam, PO Box 1738, 3000 DR, Rotterdam, The Netherlands
Curr Opin Cell Biol 15:232-40. 2003....
- Delayed and accelerated aging share common longevity assurance mechanismsBjörn Schumacher
Department of Genetics, Erasmus University Medical Center, Rotterdam, The Netherlands
PLoS Genet 4:e1000161. 2008....
- Fanconi anemia (cross)linked to DNA repairLaura J Niedernhofer
Center for Biomedical Genetics, Medical Genetic Center, Department of Cell Biology and Genetics, Erasmus Medical Center, P O Box 1738, 3000 DR Rotterdam, The Netherlands
Cell 123:1191-8. 2005..The recent discovery of two DNA helicases that, when defective, cause Fanconi anemia tips the balance in favor of the direct involvement of the FANC proteins in DNA repair and the bypass of DNA lesions...
- ERCC1 and non-small-cell lung cancerLaura J Niedernhofer
N Engl J Med 356:2538-40; author reply 2540-1. 2007
- Increased genomic instability is not a prerequisite for shortened lifespan in DNA repair deficient miceMartijn E T Dollé
National Institute of Public Health and Environment, Bilthoven, The Netherlands
Mutat Res 596:22-35. 2006....
- Deletion of the nucleotide excision repair gene Ercc1 reduces immunoglobulin class switching and alters mutations near switch recombination junctionsCarol E Schrader
Department of Molecular Genetics and Microbiology, University of Massachusetts Medical School, 55 Lake Ave North, Worcester, MA 01655, USA
J Exp Med 200:321-30. 2004..The results indicate that ERCC1 is not epistatic with MMR and suggest that ERCC1 might be involved in processing or repair of DNA lesions in S regions during CSR...
- Malondialdehyde, a product of lipid peroxidation, is mutagenic in human cellsLaura J Niedernhofer
Department of Biochemistry, Vanderbilt Institute of Chemical Biology, Center in Molecular Toxicology, Vanderbilt Ingram Comprehensive Cancer Center, Vanderbilt University School of Medicine, Nashville, Tennessee 37232, USA
J Biol Chem 278:31426-33. 2003..These experiments provide biological and biochemical evidence for the existence of MDA-induced DNA interstrand cross-links that could result from endogenous oxidative stress and likely have potent biological effects...
- DNA Interstrand Crosslinks, Cancer and AgingLaura Niedernhofer; Fiscal Year: 2007..The technique will be applied to wild type and ICL repair-defective Ercc1-/- cells to determine what proteins assemble specifically at sites of lCL damage and in what chronological order. ..
- Dietary Cancer Prevention in DNA Repair Deficient MiceLaura Niedernhofer; Fiscal Year: 2007..Finally, these experiments will reveal if ERCC1-XPF hypomorphic mice are a useful model for screening anti-oxidants that may reduce cancer risk. [unreadable] [unreadable] [unreadable]..