Niedernhofer Lab Selected Publications
Ahmad, A., Robinson, A.R., Duensing, A., van Drunen, E., Beverloo, H.B., Weisberg, D.B., Hasty, P., Hoeijmakers, J.H.J. and L.J. Niedernhofer (2008) ERCC1-XPF endonuclease facilitates DNA double-strand break repair. Mol Cell Biol., 28(16):5082-92. PMC2519706. PubMed Link
Schumacher, B., van der Pluijm, I, Moorhouse. M.J., Kosteas, T., Robinson, A.R., Suh, Y., Breit, T.M., van Steeg, H., Niedernhofer, L.J., van Ijcken, W., Bartke, A., Spindler, S.R., Hoeijmakers, J.H.J., van der Horst, G.J. and G.A. Garinis (2008) Delayed and accelerated aging share common longevity assurance mechanisms. PLoS Genet., 4(8):e1000161. PMC2493043 PubMed Link
Bhagwat, N.R., Olsen, A.L., Wang A.T., Hanada, K., Stuckert, P., Kanaar, R, D'Andrea, A., Niedernhofer, L.J.* and P.J. McHugh* (2009) XPF-ERCC1 participates in the Fanconi anemia pathway of crosslink repair. Mol Cell Biol., 29(24):6427-37. PMC2786876 *co-corresponding authors. PubMed Link
Bhagwat, N.R., Roginskaya, V.Y., Acquafondata, M.D., Dhir, R., Wood, R.D. and L.J. Niedernhofer (2009) Immunodetection of DNA repair endonuclease ERCC1-XPF in human tissue. Cancer Res., 69(17): 6831-8. PMC2739111 PubMed Link
Ahmad, A., Enzlin, J.H., Bhagwat, N., Wijgers, N., Raams, A., Appledoorn, E., Theil, A.F., Hoeijmakers, J.H.J., Vermeulen, W., Jaspers, N.G.J., Scharer, O.D*. and L.J. Niedernhofer* (2010) Mislocalization of XPF-ERCC1 nuclease contributes to reduced DNA repair in XP-F patients. PLoS Genetics, 6(3):e1000871 PMC2832669 *co-corresponding authors. PubMed Link
Hagelstrom, R.T., Blagoev, K.B., Niedernhofer, L.J., Goodwin, E.H., and S.M. Bailey (2010) Hyper- telomere recombination accelerates replicative senescence and may promote premature aging. Proc Natl Acad Sci., 107(36):15768-73. PMC2936608 PubMed Link
Orelli, B., McClendon, T.B., Tsodikov, O.V., Ellenberger, T., Niedernhofer, L.J. and O.D. Scharer (2010) The XPA-binding domain of ERCC1 is required for nucleotide excision repair but not other DNA repair pathways. J Biol Chem., 285(6):3705-12. PMC2823511 PubMed Link
Vo, N., Seo, H.-Y., Robinson, A.R., Sowa, G., Bentley, D., Taylor, L., Studer, R., Usas, A., Huard, J., Alber, S., Watkins, S.C., Lee, J., Coehlo, P., Wang, D., Loppini, M., Robbins, P.D., Niedernhofer, L.J., and J. Kang (2010) Accelerated aging of intervertebral discs in a mouse model of progeria. J Orthop Res., 28(12):1600-7. PMC3477798 PubMed Link
Choi, S., Wang, W., Ribeiro, A.J.S., Kalinowski, A., Gregg, S.Q., Opresko, P.L., Niedernhofer, L.J., Rohde, G.K. and K.N. Dahl (2011) Computational image analysis of nuclear morphology associated with various nuclear-specific aging disorders. Nucleus, 2(6):570-9. PMC3324345 PubMed Link
Dolle, M.E.T., Kuiper, R.V., Roodbergen, M., Robinson, J., de Vlugt, S., Wijnhoven, S.W.P., Beems, D., de la Fonteyne, L., de With, P., van der Pluijm, I., Niedernhofer, L,J., Hasty, P., Vijg, J., Hoeijmakers, J.H.J., and H., van Steeg (2011) Broad segmental progeroid changes in short lived Ercc1-/Δ7 mice. Pathobiology of Aging and Age-Related Diseases, 1:7219. PMC3417667 PubMed Link
Goss, J.R.*, Beer Stolz, D., Robinson, A.R., Zhang, B.S., Arbujas, N., Robbins, P.D., Glorioso, J.C. and L.J. Niedernhofer* (2011) Premature aging-related peripheral neuropathy in a mouse model of progeria. Mech Ageing Dev., 132(8-9):437-42. PMC3179831 *co-corresponding authors. PubMed Link
Vaezi, A., Wang, X-Z, Buch, S., Gooding, W., Wang, L., Seethala, R.R., Weaver, D.T., D’Andrea, A., Argiris, A., Romkes, M., Niedernhofer, L.J.* and J.R. Grandis* (2011) XPF expression correlates with clinical outcome in squamous cell carcinoma of the head and neck. Clin Cancer Res., 17(16):5513-22. PMC3156890 *co-corresponding authors. PubMed Link
Wang, D., Vo, N., Sowa, G., Hartman, R., Ngo, K., Choe, S.R., Witt, W., Dong, Q., Lee, J., Niedernhofer, L. and J. Kang (2011) Bupivacaine decreases cell viability and matrix protein synthesis in an intervertebral disc organ model system. Spine J. 11(2):139-46. PMC3056334 PubMed Link
Chen, Q., Liu, K., Robinson, A.R., Clauson, C.L., Blair, H.C. Robbins, P.D., Niedernhofer, L.J. and H. Ouyang (2012) DNA damage drives accelerated bone aging via an NF-kB-dependent mechanism. J Bone Mineral Res. Doi: 10.1002/jbmr.1851. [Epub ahead of print]. PMID: 2381008, PMC Journal – In Process. PubMed Link
Cho, J.S., Kook, S.H., Robinson, A.R., Niedernhofer, L.J.* and B.C. Lee*. (2012) Cell autonomous and non-autonomous mechanisms drive hematopoietic stem/progenitor cell loss in the absence of DNA repair. Stem cells, 31(3):511-25. PMC3582850. *co-corresponding authors. PubMed Link
DeLoia, J.A., Bhagwat, N.R., Darcy, K.M., Strange. M., Tian C., Nuttall, K., Niedernhofer, L.J., and T. Krivak (2012) Comparison of ERCC1/XPF genetic variation, mRNA and protein levels in women with advanced stage ovarian cancer treated with intraperitoneal platinum. Gynecol Oncol. 126(3):448-54. PMC3518863 PubMed Link
Gregg, S.Q., Gutierrez, V., Robinson, A.R., Woodell, T., Nakao, A., Ross, M.A., Michalopolous, G.K., Rigatti, L., Rothermel, C.E., Kamileri, I., Garinis, G.A., Beer Stolz, D., and L.J. Niedernhofer (2012) A mouse model of accelerated liver aging caused by a defect in DNA repair. Hepatology, 55(2):609-21. PMC3250572 PubMed Link
Lavasani, M., Robinson, A.R., Lu, A., Song, M., Feduska, J.M., Ahani, B., Tilstra, J.S., Feldman, C.H., Robbins, P.D., Niedernhofer, L.J.*, and J. Huard* (2012) Muscle-derived stem cell/progenitor cell dysfunction limits healthspan and lifespan in a murine progeria model. Nat Commun., 3:608. PMC3272577 *co-corresponding authors. PubMed Link
Lu, A., Proto, J., Guo, L., Tang, Y., Lavasani, M., Tilstra, J.S., Niedernhofer, L.J., Wang, B., Guttridge, D.C., Robbins, P.D. and J. Huard (2012) NF-ΚB negatively impacts the myogenic potential of muscle-derived stem cells. Mol Ther., 20(3):661-8. PMC3293604 PubMed Link
Matoka D.J., Yao, V., Harya D.S., Gregg, J.L., Robinson, A.R., Niedernhofer L.J., Parwani, A., Maier, C., and D.J. Bacich (2012) Deficiency of DNA repair nuclease ERCC1-XPF promotes prostate cancer progression in a tissue recombination model. Prostate. 72(11): 1214-22. PMC3490419 PubMed Link
Nasto, L.A., Seo, H.-Y., Robinson, A.R., Tilstra, J.S., Clauson, C.L., Sowa, G.A., Ngo, K., Dong, Q., Pola, E., Lee, J.Y., Niedernhofer, L.J., Kang, J.D., Robbins, P.D. and N.V. Vo (2012) Inhibition of NF-kB activity ameliorates age-associated disc degeneration in a mouse model of accelerated aging. Spine. 37(21):1819-1825. PMC3395770 *awarded best biology manuscript by the International Society for the Study of the Lumbar Spine (ISSLS) PubMed Link
Rubatt, J.M., Darcy, K.M., Tian, C., Muggia, F., Dhir, R., Armstrong, D.K., Bookman, M.A., Niedernhofer, L.J., Deloia, J., Birrer, M., and T.C. Krivak (2012) Pre-treatment tumor expression of ERCC1 in women with advanced stage epithelial ovarian cancer is not predictive of clinical outcomes: A Gynecologic Oncology Group study. Gynecol Oncol., 125(2):421-6. PMC3490188 PubMed Link
Smith, S.C., Robinson, A.R., Niedernhofer, L.J. and M. Hetman (2012) Down-regulation of cholesterol biosynthesis genes in the forebrain of ERCC1-deficient mice. Neurobiol Dis., 45(3):1136-44. PMC3345809 PubMed Link
Su, Y., Orelli, B., Madireddy, A., Niedernhofer, L.J. and O.D. Scharer (2012) Multiple DNA binding domains mediate the function of the ERCC1-XPF protein in nucleotide excision repair. J Biol Chem., 287(26):21846-55. PMC3381147 PubMed Link
Tilstra, J.S.*, Robinson, A.R.*, Wang, J.*, Gregg, S.Q.*, Clauson, C.L.*, Reay, D.P., Nasto, L.A., St. Croix, C.M., Usas, A., Vo, N., Huard, J., Clemens, P.R., Stoltz, D.B., Gutteridge, D.C., Watkins, S.C., Garinis, G.A., Wang, Y., Niedernhofer, L.J.*#, and P.D. Robbins# (2012) NF-kB inhibition delays DNA damage-induced senescence and aging in mice. J Clin Invest., 122(7):2601-12. *equal contributors, #co-corresponding authors. PMC3386805. PubMed Link
Wang, D., Nasto, L.A., Roughley, P., Leme, A.S., Houghton, A.M., Usas, A., Sowa, G., Lee, J., Niedernhofer, L.J., Shapiro, S., Kang, J. and N. Vo (2012) Spine degeneration in a murine model of chronic human tobacco smokers. Osteoarthritis Cartilage. 20(8):896-905. PMC3389285 PubMed Link
Wang, J., Cao, H., You, C., Yuan, B., Bahde, R., Gupta, S., Nishigori, C., Niedernhofer, L.J., Brooks, P.J., and Y. Wang (2012) Endogenous formation and repair of oxidatively induced G[8-5m]T intrastrand cross-link lesion. Nucleic Acids Res., 40(15):7368-7374. PMC3424544 PubMed Link
Wang, J., Clauson, C.L., Robbins, P.D., Niedernhofer, L.J.* and Y. Wang* (2012) The oxidative DNA lesions 8,5'-cyclopurines accumulate with aging in a tissue-specific manner. Aging Cell, 11(4):714-6. PMC3399950 *co-corresponding authors PubMed Link
Xun, Z., Rivera-Sanchez, S., Ayala-Pena, S., Lim, J., Budworth, H., Koda, E.M., Robbins, P.D., Niedernhofer, L.J., Wipf, P. and C.T. McMurray. (2012) Targeting of XJB-5-131 to mitochondria suppresses oxidative DNA damage and motor decline in a mouse model of Huntington's disease. Cell Rep., 2(5):1137-42. PMC3513647 PubMed Link
You, C., Dai, X., Yuan, B., Wang, J., Wang, J., Brooks, P.J., Niedernhofer, L.J. and Y. Wang (2012) A quantitative assay for assessing the effects of DNA lesions on transcription. Nat Chem Biol., 8:817-22. PMC3509257 PubMed Link
Gregg, S.Q., Wang, J., Maniar, S., Frantz, M.-C., Skoda, E.M., Cantu-Medellin, N., Robbins, P.D., Kelley, E.E., St. Croix, C.M., Stolz, D.B., Wang, Y., Wipf, P. and L.J. Niedernhofer (2013) Endogenous oxidative DNA lesions drive cellular senescence. J Cell Bio., (under revision).
Karakasilioti, I, Kamileri, I., Kosteas, T., Vergadi, E., Robinson, A. R., Siakouli, S., Tsatsanis, C., Niedernhofer, L.J. and G.A. Garinis, G.A. (2013) DNA damage triggers a chronic auto-inflammatory response leading to fat depletion in NER progeria. Cell Metab., (submitted).
Nasto, L.A., Robinson, A.R., Ngo, K., Clauson, C.L., Dong, Q., St. Croix, C.M., Sowa, G., Pola, E., Robbins, P.D., Kang, J., Niedernhofer, L.J.*, Wipf, P.* and N. Vo.* (2013) Mitochondrial-derived reactive oxygen species (ROS) play a causal role in aging-related intervertebral disc degeneration. J Orthop Res., 2013 Feb 6. Doi: 10.1002/jor.22320. [Epub ahead of print]. PMID: 23389888. PMC Journal-In Progress. *co-corresponding authors. PubMed Link
Nasto, L.A., Wang, D., Robinson, A.R., Clauson, C.L., Ngo, K., Dong, Q., Roughley, P., Epperly, M., Huq, S.M., Pola, E., Sowa, G., Robbins, P.D., Kang, J., Niedernhofer, L.J.*, Vo, N.* (2013) Genotoxic stress accelerates age-associated degenerative changes in intervertebral discs. Mech Ageing Dev., 134(1-2):35-42. PMC3558562. *co-corresponding authors. PubMed Link
Robinson, A.R., Tilstra, J.S., Shane, H., Gregg, S., Vo, N., Usas, A., Garinis, G. A, Wipf, P., Robbins, P.D. and L.J. Niedernhofer. (2013) A mitochondrial-targeted radical scavenger delays aging in a mouse model of human progeria, (submitted).
Roh, D.S., Du, Y., Gabriele, M., Robinson, A.R., Niedernhofer, L.J. and J.L. Funderburgh (2013) Age-related dystrophic changes in corneal endothelium from DNA repair deficient mice. Aging Cell, (under revision).
Rubatt, J.M., Beriwal, S., Niedernhofer, L.J., Kelley, J., Richard, S., Sukumvanich, P., Olawaiye, A. and T.K. Krivak (2013) BRCA1 and ERCC1 proteins expression in cervical cancer: A clinicopathologic study. Gynecol Onc., (accepted).
Tilstra, J.S., Gaddy, D.F., Dave, S.H., Niedernhofer, L.J., Plevy, S.E., and Robbins, P.D. (2013) Pharmacologic NF-κB inhibition causes antigen presenting cells to undergo ROS-dependent programmed cell death. PLoS One, (under revision).
Wipf, P., Frantz, M.-C., Skoda, E.M., Davoren, J., Wang, Z., Epperly, M., Stripay, J., Tyurin, V., Fink, B., Beyir, H., Robbins, P.D., Niedernhofer, L.J. and V. Kagan (2013) Synthesis and biochemical analysis of mitochondria-targeted nitroxide conjugates based on gramicidin S. J Amer Chem Soc. (accepted).
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