LAURA JANE NIEDERNHOFER

Summary

Affiliation: University of Pittsburgh
Country: USA

Publications

  1. pmc Mislocalization of XPF-ERCC1 nuclease contributes to reduced DNA repair in XP-F patients
    Anwaar Ahmad
    Department of Microbiology and Molecular Genetics, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania, United States of America
    PLoS Genet 6:e1000871. 2010
  2. pmc Signaling mechanisms involved in the response to genotoxic stress and regulating lifespan
    Laura 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
  3. pmc DNA repair is crucial for maintaining hematopoietic stem cell function
    Laura 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
  4. ncbi request reprint The Fanconi anemia signalosome anchor
    Laura 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
  5. pmc Nucleotide excision repair deficient mouse models and neurological disease
    Laura 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
  6. pmc Tissue-specific accelerated aging in nucleotide excision repair deficiency
    Laura 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
  7. pmc The structure-specific endonuclease Ercc1-Xpf is required to resolve DNA interstrand cross-link-induced double-strand breaks
    Laura J Niedernhofer
    Department of Cell Biology and Genetics, Erasmus Medical Center, Rotterdam, The Netherlands
    Mol Cell Biol 24:5776-87. 2004
  8. pmc ERCC1-XPF endonuclease facilitates DNA double-strand break repair
    Anwaar 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
  9. ncbi request reprint A new progeroid syndrome reveals that genotoxic stress suppresses the somatotroph axis
    Laura 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
  10. pmc Reduced hematopoietic reserves in DNA interstrand crosslink repair-deficient Ercc1-/- mice
    Joanna M Prasher
    Department of Hematology, Erasmus Medical Center, Rotterdam, The Netherlands
    EMBO J 24:861-71. 2005

Research Grants

  1. The contribution of DNA interstrand crosslinks to aging
    LAURA JANE NIEDERNHOFER; Fiscal Year: 2010
  2. The contribution of DNA interstrand crosslinks to aging
    LAURA JANE NIEDERNHOFER; Fiscal Year: 2010
  3. DNA Interstrand Crosslinks, Cancer and Aging
    Laura Niedernhofer; Fiscal Year: 2007
  4. Dietary Cancer Prevention in DNA Repair Deficient Mice
    Laura Niedernhofer; Fiscal Year: 2007

Collaborators

Detail Information

Publications21

  1. pmc Mislocalization of XPF-ERCC1 nuclease contributes to reduced DNA repair in XP-F patients
    Anwaar 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...
  2. pmc Signaling mechanisms involved in the response to genotoxic stress and regulating lifespan
    Laura 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...
  3. pmc DNA repair is crucial for maintaining hematopoietic stem cell function
    Laura 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...
  4. ncbi request reprint The Fanconi anemia signalosome anchor
    Laura 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
    ....
  5. pmc Nucleotide excision repair deficient mouse models and neurological disease
    Laura 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...
  6. pmc Tissue-specific accelerated aging in nucleotide excision repair deficiency
    Laura 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...
  7. pmc The structure-specific endonuclease Ercc1-Xpf is required to resolve DNA interstrand cross-link-induced double-strand breaks
    Laura 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...
  8. pmc ERCC1-XPF endonuclease facilitates DNA double-strand break repair
    Anwaar 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...
  9. ncbi request reprint A new progeroid syndrome reveals that genotoxic stress suppresses the somatotroph axis
    Laura 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
    ....
  10. pmc Reduced hematopoietic reserves in DNA interstrand crosslink repair-deficient Ercc1-/- mice
    Joanna 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...
  11. pmc Impaired genome maintenance suppresses the growth hormone--insulin-like growth factor 1 axis in mice with Cockayne syndrome
    Ingrid 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...
  12. pmc XPF-ERCC1 participates in the Fanconi anemia pathway of cross-link repair
    Nikhil 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...
  13. pmc Accelerated aging of intervertebral discs in a mouse model of progeria
    Nam 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...
  14. pmc 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
    Nicolaas 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...
  15. ncbi request reprint Divide and conquer: nucleotide excision repair battles cancer and ageing
    James 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
    ....
  16. pmc Delayed and accelerated aging share common longevity assurance mechanisms
    Björn Schumacher
    Department of Genetics, Erasmus University Medical Center, Rotterdam, The Netherlands
    PLoS Genet 4:e1000161. 2008
    ....
  17. ncbi request reprint Fanconi anemia (cross)linked to DNA repair
    Laura 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...
  18. ncbi request reprint ERCC1 and non-small-cell lung cancer
    Laura J Niedernhofer
    N Engl J Med 356:2538-40; author reply 2540-1. 2007
  19. ncbi request reprint Increased genomic instability is not a prerequisite for shortened lifespan in DNA repair deficient mice
    Martijn E T Dollé
    National Institute of Public Health and Environment, Bilthoven, The Netherlands
    Mutat Res 596:22-35. 2006
    ....
  20. pmc Deletion of the nucleotide excision repair gene Ercc1 reduces immunoglobulin class switching and alters mutations near switch recombination junctions
    Carol 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...
  21. ncbi request reprint Malondialdehyde, a product of lipid peroxidation, is mutagenic in human cells
    Laura 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...

Research Grants8

  1. The contribution of DNA interstrand crosslinks to aging
    LAURA JANE NIEDERNHOFER; Fiscal Year: 2010
    ..Results from these experiments will indicate if LPO promotes aging and if so, whether it does so by inducing DNA damage. These experiments will also reveal if two common industrial exposures promote aging. ..
  2. The contribution of DNA interstrand crosslinks to aging
    LAURA JANE NIEDERNHOFER; Fiscal Year: 2010
    ..Results from these experiments will indicate if LPO promotes aging and if so, whether it does so by inducing DNA damage. These experiments will also reveal if two common industrial exposures promote aging. ..
  3. DNA Interstrand Crosslinks, Cancer and Aging
    Laura 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. ..
  4. Dietary Cancer Prevention in DNA Repair Deficient Mice
    Laura Niedernhofer; Fiscal Year: 2007
    ..Similarly, the results will reveal if avoiding dietary PUFA reduces cancer risk. Finally, these experiments will reveal if ERCC1-XPF hypomorphic mice are a useful model for screening anti-oxidants that may reduce cancer risk. ..