Molecular bases of progeroid syndromes

Mitochondria, Oxidants, and Aging

Balaban, R. S., Nemoto, S., & Finkel, T. (2005) · Cell · doi:10.1016/j.cell.2005.02.001 · Cited by 4,388

Mutation of the mouse klotho gene leads to a syndrome resembling ageing

Kuro-o, M., Matsumura, Y., Aizawa, H., Kawaguchi, H., Suga, T., Utsugi, T., Ohyama, Y., Kurabayashi, M., Kaname, T., Kume, E., et al. (1997) · Nature · doi:10.1038/36285 · Cited by 3,837

A Mitochondrial Paradigm of Metabolic and Degenerative Diseases, Aging, and Cancer: A Dawn for Evolutionary Medicine

Wallace, D. C. (2005) · Annual Review of Genetics · doi:10.1146/annurev.genet.39.110304.095751 · Cited by 3,185

Premature ageing in mice expressing defective mitochondrial DNA polymerase

Trifunovic, A., Wredenberg, A., Falkenberg, M., Spelbrink, J. N., Rovio, A. T., Bruder, C. E., Bohlooly-Y, M., Gidlöf, S., Oldfors, A., Wibom, R., et al. (2004) · Nature · doi:10.1038/nature02517 · Cited by 2,734

Mitochondrial DNA mutations, oxidative stress, and apoptosis in mammalian aging

Kujoth, G. C., Hiona, A., Pugh, T. D., Someya, S., Panzer, K., Wohlgemuth, S. E., Hofer, T., Seo, A. Y., Sullivan, R., Jobling, W. A., et al. (2005) · Science · doi:10.1126/science.1112125 · Cited by 2,165

Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome

Eriksson, M., Brown, W. T., Gordon, L. B., Glynn, M. W., Singer, J., Scott, L., Erdos, M. R., Robbins, C. M., Moses, T. Y., Berglund, P., et al. (2003) · Nature · doi:10.1038/nature01629 · Cited by 1,990

Suppression of Aging in Mice by the Hormone Klotho

Kurosu, H., Yamamoto, M., Clark, J. D., Pastor, J. V., Nandi, A., Gurnani, P., McGuinness, O. P., Chikuda, H., Yamaguchi, M., Kawaguchi, H., et al. (2005) · Science · doi:10.1126/science.1112766 · Cited by 1,935

Normal oxidative damage to mitochondrial and nuclear DNA is extensive.

Richter, C., Park, J. W., & Ames, B. N. (1988) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.85.17.6465 · Cited by 1,730

Extension of murine life span by overexpression of catalase targeted to mitochondria

Schriner, S. E., Linford, N. J., Martin, G. M., Treuting, P., Ogburn, C. E., Emond, M., Coskun, P. E., Ladiges, W., Wolf, N., Van Remmen, H., et al. (2005) · Science · doi:10.1126/science.1106653 · Cited by 1,724

Human DNA Repair Genes

Wood, R., Mitchell, M., Sgouros, J., & Lindahl, T. (2001) · Science · doi:10.1126/science.1056154 · Cited by 1,719

The p66shc adaptor protein controls oxidative stress response and life span in mammals

Migliaccio, E., Giorgio, M., Mele, S., Pelicci, G., Reboldi, P., Pandolfi, P. P., Lanfrancone, L., & Pelicci, P. G. (1999) · Nature · doi:10.1038/46311 · Cited by 1,712

p53 Regulates Mitochondrial Respiration

Matoba, S., Kang, J.-G., Patino, W. D., Wragg, A., Boehm, M., Gavrilova, O., Hurley, P. J., Bunz, F., & Hwang, P. M. (2006) · Science · doi:10.1126/science.1126863 · Cited by 1,650

High levels of mitochondrial DNA deletions in substantia nigra neurons in aging and Parkinson disease

Bender, A., Krishnan, K. J., Morris, C. M., Taylor, G. A., Reeve, A. K., Perry, R. H., Jaros, E., Hersheson, J. S., Betts, J., Klopstock, T., et al. (2006) · Nature Genetics · doi:10.1038/ng1769 · Cited by 1,570

p53 mutant mice that display early ageing-associated phenotypes

Tyner, S. D., Venkatachalam, S., Choi, J., Jones, S., Ghebranious, N., Igelmann, H., Lu, X., Soron, G., Cooper, B., Brayton, C., et al. (2002) · Nature · doi:10.1038/415045a · Cited by 1,459

Lamin a truncation in Hutchinson-Gilford progeria

De Sandre-Giovannoli, A., Bernard, R., Cau, P., Navarro, C., Amiel, J., Boccaccio, I., Lyonnet, S., Stewart, C. L., Munnich, A., Le Merrer, M., et al. (2003) · Science · doi:10.1126/science.1084125 · Cited by 1,346

The plasticity of aging: insights from long-lived mutants

Kenyon, C. (2005) · Cell · doi:10.1016/j.cell.2005.02.002 · Cited by 1,338

Decline in skeletal muscle mitochondrial function with aging in humans

Short, K. R., Bigelow, M. L., Kahl, J., Singh, R., Coenen-Schimke, J., Raghavakaimal, S., & Nair, K. S. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0501559102 · Cited by 1,333

The antioxidant function of the p53 tumor suppressor

Sablina, A. A., Budanov, A. V., Ilyinskaya, G. V., Agapova, L. S., Kravchenko, J. E., & Chumakov, P. M. (2005) · Nature Medicine · doi:10.1038/nm1320 · Cited by 1,137

Lamin A-dependent nuclear defects in human aging

Scaffidi, P., & Misteli, T. (2006) · Science · doi:10.1126/science.1127168 · Cited by 1,125

Calorie restriction promotes mitochondrial biogenesis by inducing the expression of eNOS

Nisoli, E., Tonello, C., Cardile, A., Cozzi, V., Bracale, R., Tedesco, L., Falcone, S., Valerio, A., Cantoni, O., Clementi, E., et al. (2005) · Science · doi:10.1126/science.1117728 · Cited by 1,107

Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson–Gilford progeria syndrome

Goldman, R. D., Shumaker, D. K., Erdos, M. R., Eriksson, M., Goldman, A. E., Gordon, L. B., Gruenbaum, Y., Khuon, S., Mendez, M., Varga, R., et al. (2004) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0402943101 · Cited by 1,103

Calorie restriction, SIRT1 and metabolism: understanding longevity

Bordone, L., & Guarente, L. (2005) · Nature Reviews Molecular Cell Biology · doi:10.1038/nrm1616 · Cited by 1,038

DNA Repair, Genome Stability, and Aging

Lombard, D. B., Chua, K. F., Mostoslavsky, R., Franco, S., Gostissa, M., & Alt, F. W. (2005) · Cell · doi:10.1016/j.cell.2005.01.028 · Cited by 944

Mitochondrial DNA deletions are abundant and cause functional impairment in aged human substantia nigra neurons

Kraytsberg, Y., Kudryavtseva, E., McKee, A. C., Geula, C., Kowall, N. W., & Khrapko, K. (2006) · Nature Genetics · doi:10.1038/ng1778 · Cited by 896

Cockayne syndrome: Review of 140 cases

Nance, M. A., & Berry, S. A. (1992) · American Journal of Medical Genetics · doi:10.1002/ajmg.1320420115 · Cited by 806

Calorie restriction–the SIR2 connection

Guarente, L., & Picard, F. (2005) · Cell · doi:10.1016/j.cell.2005.01.029 · Cited by 793

Differential induction of transcriptionally active p53 following UV or lonizing radiation: Defects in chromosome instability syndromes?

Lü, X. (1993) · Cell · doi:10.1016/0092-8674(93)90496-d · Cited by 773

RecQ helicases: caretakers of the genome

Hickson, I. D. (2003) · Nature reviews. Cancer · doi:10.1038/nrc1012 · Cited by 680

Genomic instability in laminopathy-based premature aging

Liu, B., Wang, J., Chan, K. M., Tjia, W. M., Deng, W., Guan, X., Huang, J.-D., Li, K. M., Chau, P. Y., Chen, D. J., et al. (2005) · Nature Medicine · doi:10.1038/nm1266 · Cited by 650

Molecular Mechanisms of Mammalian Global Genome Nucleotide Excision Repair

Gillet, L. C. J., & Schärer, O. D. (2006) · Chemical Reviews · doi:10.1021/cr040483f · Cited by 642

Nuclear Lamins: Laminopathies and Their Role in Premature Ageing

Broers, J. L. V., Ramaekers, F. C. S., Bonne, G., Yaou, R. B., & Hutchison, C. J. (2006) · Physiological Reviews · doi:10.1152/physrev.00047.2005 · Cited by 587

Hutchinson–Gilford progeria syndrome: Review of the phenotype

Hennekam, R. C. M. (2006) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.31346 · Cited by 587

Sirtuins: Sir2-related NAD-dependent protein deacetylases.

North, B. J., & Verdin, E. M. (2004) · Genome biology · doi:10.1186/gb-2004-5-5-224 · Cited by 586

Somatic mtDNA mutations cause aging phenotypes without affecting reactive oxygen species production

Trifunovic, A., Hansson, A., Wredenberg, A., Rovio, A. T., Dufour, E., Khvorostov, I., Spelbrink, J. N., Wibom, R., Jacobs, H. T., & Larsson, N.-G. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0508886102 · Cited by 555

Defective prelamin A processing and muscular and adipocyte alterations in Zmpste24 metalloproteinase–deficient mice

Pendás, A. M., Zhou, Z., Cadiñanos, J., Freije, J. M. P., Wang, J., Hultenby, K., Astudillo, A., Wernerson, A., Rodríguez, F., Tryggvason, K., et al. (2002) · Nature Genetics · doi:10.1038/ng871 · Cited by 554

Reversal of the cellular phenotype in the premature aging disease Hutchinson-Gilford progeria syndrome

Scaffidi, P., & Misteli, T. (2005) · Nature Medicine · doi:10.1038/nm1204 · Cited by 553

Aging and Genome Maintenance: Lessons from the Mouse?

Hasty, P., Campisi, J., Hoeijmakers, J., van Steeg, H., & Vijg, J. (2003) · Science · doi:10.1126/science.1079161 · Cited by 553

Mandibuloacral Dysplasia Is Caused by a Mutation in LMNA-Encoding Lamin A/C

Novelli, G., Muchir, A., Sangiuolo, F. C., Helbling-Leclerc, A., D’Apice, M. R., Massart, C., Capon, F., Sbraccia, P., Federici, M., Lauro, R., et al. (2002) · The American Journal of Human Genetics · doi:10.1086/341908 · Cited by 550

DNA repair-deficient diseases, xeroderma pigmentosum, Cockayne syndrome and trichothiodystrophy

Lehmann, A. (2003) · Biochimie · doi:10.1016/j.biochi.2003.09.010 · Cited by 518

The Cockayne syndrome group A gene encodes a WD repeat protein that interacts with CSB protein and a subunit of RNA polymerase II TFIIH

Henning, K. A., Li, L., Iyer, N., McDaniel, L. D., Reagan, M. S., Legerski, R., Schultz, R. A., Stefanini, M., Lehmann, A. R., Mayne, L. V., et al. (1995) · Cell · doi:10.1016/0092-8674(95)90028-4 · Cited by 507

Farnesyl Pyrophosphate Synthase Is the Molecular Target of Nitrogen-Containing Bisphosphonates

van Beek, E. R., Pieterman, E. J., Cohen, L., Löwik, C. W. G. M., & Papapoulos, S. E. (1999) · Biochemical and Biophysical Research Communications · doi:10.1006/bbrc.1999.1499 · Cited by 496

Accelerated ageing in mice deficient in Zmpste24 protease is linked to p53 signalling activation

Varela, I., Cadiñanos, J., Pendás, A. M., Gutiérrez-Fernández, A., Folgueras, A. R., Sánchez, L. M., Zhou, Z., Rodríguez, F. J., Stewart, C. L., Vega, J. A., et al. (2005) · Nature · doi:10.1038/nature04019 · Cited by 466

Spindle Multipolarity Is Prevented by Centrosomal Clustering

Quintyne, N. J., Reing, J. E., Hoffelder, D. R., Gollin, S. M., & Saunders, W. S. (2005) · Science · doi:10.1126/science.1104905 · Cited by 446

Zmpste24 deficiency in mice causes spontaneous bone fractures, muscle weakness, and a prelamin A processing defect

Bergö, M. O., Gavino, B. J., Ross, J., Schmidt, W. K., Hong, C., Kendall, L. V., Mohr, A., Meta, M. D., Genant, H. K., Jiang, Y., et al. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.192460799 · Cited by 443

The comings and goings of nucleotide excision repair factors on damaged DNA

Riedl, T., Hanaoka, F., & Egly, J. (2003) · The EMBO Journal · doi:10.1093/emboj/cdg489 · Cited by 439

A p53-p66Shc signalling pathway controls intracellular redox status, levels of oxidation-damaged DNA and oxidative stress-induced apoptosis

Trinei, M., Giorgio, M., Cicalese, A., Barozzi, S., Ventura, A., Migliaccio, E., Milia, E., Padura, I. M., Raker, V. A., Maccarana, M., et al. (2002) · Oncogene · doi:10.1038/sj.onc.1205513 · Cited by 436

Human DNA repair genes, 2005

Wood, R. D., Mitchell, M., & Lindahl, T. L. (2005) · Mutation research. Fundamental and molecular mechanisms of mutagenesis · doi:10.1016/j.mrfmmm.2005.03.007 · Cited by 428

Cancer in xeroderma pigmentosum and related disorders of DNA repair

Cleaver, J. E. (2005) · Nature Reviews Cancer · doi:10.1038/nrc1652 · Cited by 427

Telomere-binding Protein TRF2 Binds to and Stimulates the Werner and Bloom Syndrome Helicases

Opresko, P. L., von Kobbe, C., Lainé, J., Harrigan, J. A., Hickson, I. D., & Bohr, V. A. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m205396200 · Cited by 409

Zinc metalloproteinase, ZMPSTE24, is mutated in mandibuloacral dysplasia

Agarwal, A. K. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg213 · Cited by 400

Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome

Capell, B. C., Erdos, M. R., Madigan, J. P., Fiordalisi, J. J., Varga, R., Conneely, K. N., Gordon, L. B., Der, C. J., Cox, A. D., & Collins, F. S. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0506001102 · Cited by 395

Lamin A and ZMPSTE24 (FACE-1) defects cause nuclear disorganization and identify restrictive dermopathy as a lethal neonatal laminopathy

Navarro, C. L., Sandre‐Giovannoli, A. D., Bernard, R., Boccaccio, I., Boyer, A., Geneviève, D., Hadj-Rabia, S., Gaudy‐Marqueste, C., Smitt, S., Vabres, P., et al. (2004) · Human Molecular Genetics · doi:10.1093/hmg/ddh265 · Cited by 377

Systematic identification of human mitochondrial disease genes through integrative genomics

Calvo, S. E., Jain, M., Xie, X. S., Sheth, S. A., Chang, B., Goldberger, O., Spinazzola, A., Zeviani, M., Carr, S. A., & Mootha, V. K. (2006) · Nature Genetics · doi:10.1038/ng1776 · Cited by 352

UV-induced ubiquitination of RNA polymerase II: a novel modification deficient in Cockayne syndrome cells.

Bregman, D. B., Halaban, R., van Gool, A. J., Henning, K. A., Friedberg, E. C., & Warren, S. L. (1996) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.93.21.11586 · Cited by 331

Diabetes Promotes Cardiac Stem Cell Aging and Heart Failure, Which Are Prevented by Deletion of the p66 shc Gene

Rota, M., LeCapitaine, N., Hosoda, T., Boni, A., De Angelis, A., Padin-Iruegas, M. E., Esposito, G., Vitale, S., Urbanek, K., Casarsa, C., et al. (2006) · Circulation Research · doi:10.1161/01.res.0000231289.63468.08 · Cited by 330

Ku complex interacts with and stimulates the Werner proteinPreprint

Cooper, M. P., Machwe, A., Orren, D. K., Brosh, R. M., Ramsden, D. A., & Bohr, V. A. (2000) · Genes & Development · doi:10.1101/gad.14.8.907 · Cited by 320

A Protein Farnesyltransferase Inhibitor Ameliorates Disease in a Mouse Model of Progeria

Fong, L. G., Frost, D. J., Meta, M. D., Qiao, X., Yang, S. H., Coffinier, C., & Young, S. G. (2006) · Science · doi:10.1126/science.1124875 · Cited by 315

Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson–Gilford progeria syndrome mutation

Yang, S. H., Bergö, M. O., Toth, J. I., Qiao, X., Hu, Y., Sandoval, S., Meta, M. D., Bendale, P. M., Gelb, M. H., Young, S. G., et al. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0504641102 · Cited by 311

Increased activity of p53 in senescing fibroblasts

Atadja, P., Wong, H., Garkavtsev, I., Veillette, C., & Riabowol, K. (1995) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.92.18.8348 · Cited by 308

Minireview: Role of the Growth Hormone/Insulin-Like Growth Factor System in Mammalian Aging

Bartke, A. (2005) · Endocrinology · doi:10.1210/en.2005-0411 · Cited by 307

Functional and Physical Interaction between WRN Helicase and Human Replication Protein A

Brosh, R. M., Orren, D. K., Nehlin, J. O., Ravn, P., Kenny, M. K., Machwe, A., & Bohr, V. A. (1999) · Journal of Biological Chemistry · doi:10.1074/jbc.274.26.18341 · Cited by 306

Loss of ZMPSTE24 (FACE-1) causes autosomal recessive restrictive dermopathy and accumulation of Lamin A precursors

Navarro, C. L., Cadiñanos, J., Sandre‐Giovannoli, A. D., Bernard, R., Courrier, S., Boccaccio, I., Boyer, A., Kleijer, W. J., Wagner, A., Giuliano, F., et al. (2005) · Human Molecular Genetics · doi:10.1093/hmg/ddi159 · Cited by 299

Damage recognition in nucleotide excision repair of DNA

Batty, D. P., & Wood, R. D. (2000) · Gene · doi:10.1016/s0378-1119(99)00489-8 · Cited by 298

Thematic review series: Lipid Posttranslational Modifications. Farnesyl transferase inhibitors

Basso, A. D., Kirschmeier, P. T., & Bishop, W. R. (2005) · Journal of Lipid Research · doi:10.1194/jlr.r500012-jlr200 · Cited by 297

Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromes

Toth, J. I., Yang, S. H., Qiao, X., Beigneux, A. P., Gelb, M. H., Moulson, C. L., Miner, J. H., Young, S. G., & Fong, L. G. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0505767102 · Cited by 292

Age-related changes of nuclear architecture in Caenorhabditis elegans

Haithcock, E., Dayani, Y., Neufeld, E., Zahand, A. J., Feinstein, N., Mattout, A., Gruenbaum, Y., & Liu, J. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0506955102 · Cited by 292

The Life Span Determinant p66Shc Localizes to Mitochondria Where It Associates with Mitochondrial Heat Shock Protein 70 and Regulates Trans-membrane Potential

Orsini, F., Migliaccio, E., Moroni, M., Contursi, C., Raker, V. A., Piccini, D., Martín-Padura, I., Pelliccia, G., Trinei, M., Bono, M., et al. (2004) · Journal of Biological Chemistry · doi:10.1074/jbc.m401844200 · Cited by 291

A Mitotic Lamin B Matrix Induced by RanGTP Required for Spindle Assembly

Tsai, M.-Y., Wang, S., Heidinger-Pauli, J. M., Shumaker, D. K., Adam, S. A., Goldman, R. D., & Zheng, Y. (2006) · Science · doi:10.1126/science.1122771 · Cited by 290

Novel role of p53 in maintaining mitochondrial genetic stability through interaction with DNA Pol γ

Achanta, G., Sasaki, R., Li, F., Carew, J. S., Lu, W., Pelicano, H., Keating, M. J., & Huang, P. (2005) · The EMBO Journal · doi:10.1038/sj.emboj.7600819 · Cited by 285

Nitric oxide and mitochondrial biogenesis

Nisoli, E., & Carruba, M. O. (2006) · Journal of Cell Science · doi:10.1242/jcs.03062 · Cited by 285

Bisphosphonates used for the treatment of bone disorders inhibit squalene synthase and cholesterol biosynthesis.

Amin, D., Cornell, S., Gustafson, S., Needle, S., Ullrich, J., Bilder, G., & Perrone, M. H. (1992) · Journal of Lipid Research · doi:10.1016/s0022-2275(20)41388-4 · Cited by 281

Minireview: The Role of Oxidative Stress in Relation to Caloric Restriction and Longevity

Gredilla, R., & Barja, G. (2005) · Endocrinology · doi:10.1210/en.2005-0378 · Cited by 269

Incomplete processing of mutant lamin A in Hutchinson–Gilford progeria leads to nuclear abnormalities, which are reversed by farnesyltransferase inhibition

Glynn, M. W., & Glover, T. W. (2005) · Human Molecular Genetics · doi:10.1093/hmg/ddi326 · Cited by 259

Crystal structure of human p32, a doughnut-shaped acidic mitochondrial matrix protein

Jiang, J., Zhang, Y., Krainer, A. R., & Xu, R.-M. (1999) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.96.7.3572 · Cited by 253

The Werner Syndrome Gene Product Co-purifies with the DNA Replication Complex and Interacts with PCNA and Topoisomerase I

Lebel, M., Spillare, E. A., Harris, C. C., & Leder, P. (1999) · Journal of Biological Chemistry · doi:10.1074/jbc.274.53.37795 · Cited by 246

RecQ DNA helicase is a suppressor of illegitimate recombination in Escherichia coli

Hanada, K., Ukita, T., Kohno, Y., Saito, K., Kato, J., & Ikeda, H. (1997) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.94.8.3860 · Cited by 246

Nuclear envelope disorganization in fibroblasts from lipodystrophic patients with heterozygous R482Q/W mutations in the lamin A/C gene

Vigouroux, C., Auclair, M., Dubosclard, E., Pouchelet, M., Capeau, J., Courvalin, J., & Buendia, B. (2001) · Journal of Cell Science · doi:10.1242/jcs.114.24.4459 · Cited by 239

The spectrum ofWRNmutations in Werner syndrome patients

Huang, S., Lee, L., Hanson, N. B., Lenaerts, C., Hoehn, H., Poot, M., Rubin, C. D., Chen, D.-F., Yang, C., Juch, H., et al. (2006) · Human Mutation · doi:10.1002/humu.20337 · Cited by 234

REACTIVE OXYGEN INTERMEDIATES IN BIOCHEMISTRY

Naqui, A., Chance, B., & Cadenas, E. (1986) · Annual Review of Biochemistry · doi:10.1146/annurev.bi.55.070186.001033 · Cited by 234

Mechanisms of Protein Import into Mitochondria

Truscott, K. N., Brandner, K., & Pfanner, N. (2003) · Current Biology · doi:10.1016/s0960-9822(03)00239-2 · Cited by 225

Congenital Absence of Hair and Mammary Glands with Atrophic Condition of the Skin and its Appendages in a Boy Whose Mother Had Been Almost Wholly Bald from Alopecia Areata from the Age of Six

Hutchinson, J. (1886) · Journal of the Royal Society of Medicine · doi:10.1177/095952878606900127 · Cited by 215

Inhibiting farnesylation reverses the nuclear morphology defect in a HeLa cell model for Hutchinson-Gilford progeria syndrome

Mallampalli, M. P., Huyer, G., Bendale, P. M., Gelb, M. H., & Michaelis, S. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0503712102 · Cited by 210

Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24 -deficient mice

Fong, L. G., Ng, J. K., Meta, M. D., Coté, N., Yang, S. H., Stewart, C. L., Sullivan, T., Burghardt, A. J., Majumdar, S., Reue, K., et al. (2004) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0408558102 · Cited by 209

Nucleoplasmic localization of prelamin A: implications for prenylation-dependent lamin A assembly into the nuclear lamina.

Lutz, R. J., Trujillo, M. A., Denham, K. S., Wenger, L., & Sinensky, M. S. (1992) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.89.7.3000 · Cited by 196

The mitochondrial DNA polymerase as a target of oxidative damage

Graziewicz, M. A. (2002) · Nucleic Acids Research · doi:10.1093/nar/gkf392 · Cited by 187

Functional interaction between the Werner Syndrome protein and DNA polymerase δ

Kamath‐Loeb, A. S., Johansson, E., Burgers, P., & Loeb, L. A. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.97.9.4603 · Cited by 186

Transcription-coupled and DNA damage-dependent ubiquitination of RNA polymerase II in vitro

Lee, K., Wang, D., Lippard, S. J., & Sharp, P. A. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.072068399 · Cited by 182

Physical and Functional Interaction between p53 and the Werner's Syndrome Protein

Blander, G., Kipnis, J., Martínez-Leal, J. F., Yu, C., Schellenberg, G. D., & Oren, M. (1999) · Journal of Biological Chemistry · doi:10.1074/jbc.274.41.29463 · Cited by 181

p53-Mediated apoptosis is attenuated in Werner syndrome cellsPreprint

Spillare, E. A., Robles, A. I., Wang, X. W., Shen, J.-C., Yu, C., Schellenberg, G. D., & Harris, C. C. (1999) · Genes & Development · doi:10.1101/gad.13.11.1355 · Cited by 178

Some HIV protease inhibitors alter lamin A/C maturation and stability, SREBP-1 nuclear localization and adipocyte differentiation

Caron, M., Auclair, M., Sterlingot, H., Kornprobst, M., & Capeau, J. (2003) · AIDS · doi:10.1097/00002030-200311210-00005 · Cited by 176

Aging of Hutchinson–Gilford progeria syndrome fibroblasts is characterised by hyperproliferation and increased apoptosis

Bridger, J. M., & Kill, I. R. (2004) · Experimental Gerontology · doi:10.1016/j.exger.2004.02.002 · Cited by 176

Differential inhibitory effects of lovastatin on protein isoprenylation and sterol synthesis.

Sinensky, M. S., Beck, L. A., Leonard, S. S., & Evans, R. (1990) · Journal of Biological Chemistry · doi:10.1016/s0021-9258(17)45464-0 · Cited by 172

The emerging therapeutic potential of sirtuin-interacting drugs: from cell death to lifespan extension

Porcu, M., & Chiarugi, A. (2004) · Trends in Pharmacological Sciences · doi:10.1016/j.tips.2004.12.009 · Cited by 172

Nuclear lamins, diseases and aging

Mattout, A., Dechat, T., Adam, S. A., Goldman, R. D., & Gruenbaum, Y. (2006) · Current Opinion in Cell Biology · doi:10.1016/j.ceb.2006.03.007 · Cited by 171

The splicing factor‐associated protein, p32, regulates RNA splicing by inhibiting ASF/SF2 RNA binding and phosphorylation

Petersen‐Mahrt, S. K., Nilsson, C. E., Öhrmalm, C., Matthews, D. A., Russell, W. C., & Akusjärvi, G. (1999) · The EMBO Journal · doi:10.1093/emboj/18.4.1014 · Cited by 171

Molecular Analysis of Mutations in the CSB(ERCC6) Gene in Patients with Cockayne Syndrome

Mallery, D. L., Tanganelli, B., Colella, S., Steingrimsdottir, H., van Gool, A. J., Troelstra, C., Stefanini, M., & Lehmann, A. R. (1998) · The American Journal of Human Genetics · doi:10.1086/301686 · Cited by 161

Increased p53 Protein Associated with Aging in Human Diploid Fibroblasts

Kulju, K. S., & Lehman, J. M. (1995) · Experimental Cell Research · doi:10.1006/excr.1995.1095 · Cited by 155

Instabilotyping: comprehensive identification of frameshift mutations caused by coding region microsatellite instability.

Mori, Y., Yin, J., Rashid, A., Leggett, B., Young, J. P., Simms, L. A., Kuehl, P. M., LANGENBERG, P. W., Meltzer, S. J., & Stine, O. C. (2001) · PubMed · Cited by 150

Transcriptional Blockade Induces p53-dependent Apoptosis Associated with Translocation of p53 to Mitochondria

Arima, Y., Nitta, M., Kuninaka, S., Zhang, D., Fujiwara, T., Taya, Y., Nakao, M., & Saya, H. (2005) · Journal of Biological Chemistry · doi:10.1074/jbc.m410691200 · Cited by 149

The Mammalian Longevity-associated Gene Product p66 Regulates Mitochondrial Metabolism

Nemoto, S., Combs, C. A., French, S. A., Ahn, B., Fergusson, M. M., Balaban, R. S., & Finkel, T. (2006) · Journal of Biological Chemistry · doi:10.1074/jbc.m511626200 · Cited by 147

Reactive Oxygen Species–Mediated Mitochondria-to-Nucleus Signaling: A Key to Aging and Radical-Caused Diseases

Störz, P. (2006) · Science s STKE · doi:10.1126/stke.3322006re3 · Cited by 147

Energy- and temperature-dependent transport of integral proteins to the inner nuclear membrane via the nuclear pore

Ohba, T., Schirmer, E. C., Nishimoto, T., & Gerace, L. (2004) · The Journal of Cell Biology · doi:10.1083/jcb.200409149 · Cited by 141

Colocalization, Physical, and Functional Interaction between Werner and Bloom Syndrome Proteins

von Kobbe, C., Karmakar, P., Dawut, L., Opresko, P. L., Zeng, X., Brosh, R. M., Hickson, I. D., & Bohr, V. A. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m200914200 · Cited by 139

Molecular clues into the pathogenesis of statin‐mediated muscle toxicity

Baker, S. K. (2005) · Muscle & Nerve · doi:10.1002/mus.20291 · Cited by 137

Effects of HIV protease inhibitor therapy on lipid metabolism

Hui, D. Y. (2003) · Progress in Lipid Research · doi:10.1016/s0163-7827(02)00046-2 · Cited by 135

TRF2 recruits the Werner syndrome (WRN) exonuclease for processing of telomeric DNA

Machwe, A., Xiao, L., & Orren, D. K. (2004) · Oncogene · doi:10.1038/sj.onc.1206906 · Cited by 130

The Werner syndrome protein operates in base excision repair and cooperates with DNA polymerase

Harrigan, J. A. (2006) · Nucleic Acids Research · doi:10.1093/nar/gkj475 · Cited by 127

WRN Interacts Physically and Functionally with the Recombination Mediator Protein RAD52

Baynton, K., Otterlei, M., Bjørås, M., von Kobbe, C., Bohr, V. A., & Seeberg, E. C. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.m303885200 · Cited by 122

Alterations of nuclear envelope and chromatin organization in mandibuloacral dysplasia, a rare form of laminopathy

Filesi, I., Gullotta, F., Lattanzi, G., D’Apice, M. R., Capanni, C., Nardone, A. M., Columbaro, M., Scarano, G., Mattioli, E., Sabatelli, P., et al. (2005) · Physiological Genomics · doi:10.1152/physiolgenomics.00060.2005 · Cited by 122

The Werner Syndrome Protein Stimulates DNA Polymerase β Strand Displacement Synthesis via Its Helicase Activity

Harrigan, J. A., Opresko, P. L., von Kobbe, C., Kedar, P. S., Prasad, R., Wilson, S. H., & Bohr, V. A. (2003) · Journal of Biological Chemistry · doi:10.1074/jbc.m213103200 · Cited by 121

Compound heterozygous ZMPSTE24 mutations reduce prelamin A processing and result in a severe progeroid phenotype

Shackleton, S., Smallwood, D. T., Clayton, P. T., Wilson, L. C., Agarwal, A. K., Garg, A., & Trembath, R. C. (2005) · Journal of Medical Genetics · doi:10.1136/jmg.2004.029751 · Cited by 120

Mechanisms of DNA damage recognition and strand discrimination in human nucleotide excision repair

Dip, R., Camenisch, U., & Naegeli, H. (2004) · DNA repair · doi:10.1016/j.dnarep.2004.05.005 · Cited by 118

Savior and slayer: the two faces of p53

Bensaad, K., & Vousden, K. H. (2005) · Nature Medicine · doi:10.1038/nm1205-1278 · Cited by 113

The HIV-1 nucleoside reverse transcriptase inhibitors stavudine and zidovudine alter adipocyte functions in vitro

Caron, M., Auclair, M., Lagathu, C., Lombès, A., Walker, U. A., Kornprobst, M., & Capeau, J. (2004) · AIDS · doi:10.1097/00002030-200411050-00004 · Cited by 107

Expression of Lamin A Mutated in the Carboxyl-Terminal Tail Generates an Aberrant Nuclear Phenotype Similar to That Observed in Cells from Patients with Dunnigan-Type Partial Lipodystrophy and Emery-Dreifuss Muscular Dystrophy

Favreau, C. (2003) · Experimental Cell Research · doi:10.1006/excr.2002.5669 · Cited by 105

Understanding aging: revealing order out of chaos

Dufour, É., & Larsson, N. (2004) · Biochimica et Biophysica Acta (BBA) - Bioenergetics · doi:10.1016/j.bbabio.2004.04.020 · Cited by 103

A Role for p53 in Maintaining and Establishing the Quiescence Growth Arrest in Human Cells

Itahana, K., Dimri, G. P., Hara, E., Itahana, Y., Zou, Y., Desprez, P., & Campisi, J. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m201028200 · Cited by 100

Compound heterozygosity for mutations in LMNA causes a progeria syndrome without prelamin A accumulation

Verstraeten, V. L. R. M. (2006) · Human Molecular Genetics · doi:10.1093/hmg/ddl172 · Cited by 100

Mechanism of Aminobisphosphonate Action: Characterization of Alendronate Inhibition of the Isoprenoid Pathway

Keller, R. K., & Fliesler, S. J. (1999) · Biochemical and Biophysical Research Communications · doi:10.1006/bbrc.1999.1849 · Cited by 89

Role of NuMA in vertebrate cells: review of an intriguing multifunctional protein

Sun, Q. (2006) · Frontiers in bioscience · doi:10.2741/1868 · Cited by 85

The HIV Protease Inhibitors Nelfinavir and Saquinavir, but Not a Variety of HIV Reverse Transcriptase Inhibitors, Adversely Affect Human Proteasome Function

Piccinini, M., RINAUDO, M. T., Anselmino, A., Buccinnà, B., Ramondetti, C., Dematteis, A., Ricotti, E., Palmisano, L., Mostert, M., & Tovo, P. (2005) · Antiviral Therapy · doi:10.1177/135965350501000203 · Cited by 84

LMNA mutation in a 45 year old Japanese subject with Hutchinson-Gilford progeria syndrome

Fukuchi, K., Katsuya, T., Sugimoto, K., Kuremura, M., Kim, H. D., Li, L., & Ogihara, T. (2004) · Journal of Medical Genetics · doi:10.1136/jmg.2003.014688 · Cited by 82

p53 Moves to Mitochondria: A Turn on the Path to Apoptosis

Murphy, M. E., Leu, J. I.-J., & George, D. L. (2004) · Cell Cycle · doi:10.4161/cc.3.7.956 · Cited by 75

Making ends meet in old age: DSB repair and aging

Gorbunova, V. A., & Seluanov, A. (2005) · Mechanisms of Ageing and Development · doi:10.1016/j.mad.2005.02.008 · Cited by 74

Temporal Association of Protamine 1 with the Inner Nuclear Membrane Protein Lamin B Receptor during Spermiogenesis

Mylonis, I., Drosou, V., Brancorsini, S., Nikolakaki, E., Sassone‐Corsi, P., & Giannakouŕos, T. (2004) · Journal of Biological Chemistry · doi:10.1074/jbc.m311949200 · Cited by 74

Klotho-induced insulin resistance: a blessing in disguise?

Unger, R. H. (2006) · Nature Medicine · doi:10.1038/nm0106-56 · Cited by 71

Transcription-coupled repair and premature ageing

Andressoo, J., & Hoeijmakers, J. H. J. (2005) · Mutation research. Fundamental and molecular mechanisms of mutagenesis · doi:10.1016/j.mrfmmm.2005.04.004 · Cited by 70

The protein shuffle

Park, C., & Choi, B. (2006) · FEBS Journal · doi:10.1111/j.1742-4658.2006.05189.x · Cited by 65

The lamin B receptor‐associated protein p34 shares sequence homology and antigenic determinants with the splicing factor 2‐associated protein p32

Simos, G., & Georgatos, S. D. (1994) · FEBS Letters · doi:10.1016/0014-5793(94)00479-x · Cited by 65

Restrictive Dermopathy Report of 12 Cases

Smitt, J. H. S. (1998) · Archives of Dermatology · doi:10.1001/archderm.134.5.577 · Cited by 62

IGF-1 signaling and aging

Holzenberger, M., Kappeler, L., & Filho, C. D. D. M. (2004) · Experimental Gerontology · doi:10.1016/j.exger.2004.08.017 · Cited by 61

Cell and Molecular Biology of Spindle Poles and NuMA

Fant, X., Merdes, A., & Haren, L. (2004) · International review of cytology · doi:10.1016/s0074-7696(04)38001-0 · Cited by 59

Genetic alterations in accelerated ageing syndromes

Puzianowska‐Kuźnicka, M., & Kuźnicki, J. (2004) · The International Journal of Biochemistry & Cell Biology · doi:10.1016/j.biocel.2004.10.011 · Cited by 54

Murine Models of Life Span Extension

Quarrie, J. K., & Riabowol, K. T. (2004) · Science of Aging Knowledge Environment · doi:10.1126/sageke.2004.31.re5 · Cited by 53

Mitochondrial Protein p32 Can Accumulate in the Nucleus

Brokstad, K. A., Kalland, K., Russell, W. C., & Matthews, D. A. (2001) · Biochemical and Biophysical Research Communications · doi:10.1006/bbrc.2001.4473 · Cited by 52

Farnesyl and Geranylgeranyl Transferase Inhibitors Induce G1 Arrest by Targeting the Proteasome

Efuet, E. T., & Keyomarsi, K. (2006) · Cancer Research · doi:10.1158/0008-5472.can-05-3416 · Cited by 50

Possible Ways Nucleoside Analogues Can Affect Mitochondrial Dna Content and Gene Expression during HIV Therapy

Côté, H. C. F. (2005) · Antiviral Therapy · doi:10.1177/135965350501002s02 · Cited by 48

Progeria Infantum (Hutchinson–Gilford Syndrome) Associated with Scleroderma‐Like Lesions and Acro‐Osteolysis: A Case Report and Brief Review of the Literature

Jansen, T., & Romiti, R. (2000) · Pediatric Dermatology · doi:10.1046/j.1525-1470.2000.01775.x · Cited by 47

Interstitial chromatin alteration causes persistent p53 activation involved in the radiation-induced senescence-like growth arrest

Suzuki, M., Suzuki, K., Kodama, S., & Watanabe, M. (2005) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2005.11.167 · Cited by 42

Restrictive Dermopathy: A Case Report and a Critical Review of All Hypotheses of its Origin

Nijsten, T. E. C., Moor, A. D., Colpaert, C., Robert, K., Mahieu, L. M., & Lambert, J. (2002) · Pediatric Dermatology · doi:10.1046/j.1525-1470.2002.00029.x · Cited by 42

Human diseases deficient in RecQ helicases

Harrigan, J. A. (2003) · Biochimie · doi:10.1016/j.biochi.2003.10.006 · Cited by 40

Restrictive dermopathy: report of a case and review of the literature

Wesche, W. A., Cutlan, R. T., Khare, V. K., Chesney, T. M., & Shanklin, D. R. (2001) · Journal of Cutaneous Pathology · doi:10.1034/j.1600-0560.2001.028004211.x · Cited by 38

On Cataract in Conjunction with Scleroderma

Werner, O. (1985) · Advances in experimental medicine and biology · doi:10.1007/978-1-4684-7853-2_1 · Cited by 37

Human p32, interacts with B subunit of the CCAAT-binding factor, CBF/NF-Y, and inhibits CBF-mediated transcription activation in vitro

Chattopadhyay, C. (2004) · Nucleic Acids Research · doi:10.1093/nar/gkh692 · Cited by 35

From Immature Lamin to Premature Aging: Molecular Pathways and Therapeutic Opportunities

Cadiñanos, J., Varela, I., López-Otı́n, C., & Freije, J. M. P. (2005) · Cell Cycle · doi:10.4161/cc.4.12.2202 · Cited by 33

Mice and mitochondria

Martin, G. M., & Loeb, L. A. (2004) · Nature · doi:10.1038/429357a · Cited by 30

Role of sirtuin proteins in life extension by caloric restriction

Masoro, E. J. (2004) · Mechanisms of Ageing and Development · doi:10.1016/j.mad.2004.08.011 · Cited by 30

In vitro evidence of inhibition of mitochondrial protease processing by HIV-1 protease inhibitors in yeast: a possible contribution to lipodystrophy syndrome

Mukhopadhyay, A. (2002) · Mitochondrion · doi:10.1016/s1567-7249(02)00042-9 · Cited by 29

Role of p53 tumor suppressor in ageing: regulation of transient cell cycle arrest and terminal senescence.

Węsierska‐Gądek, J., Wojciechowski, J., Ranftler, C., & Schmid, G. (2005) · PubMed · Cited by 22

RNA polymerase II stalled on a DNA template during transcription elongation is ubiquitinated and the ubiquitination facilitates displacement of the elongation complex.

Yang, L. Y., Jiang, H., & Rangel, K. (2003) · PubMed · Cited by 18

Civil Medical Organization in War

Ward, R. O. (1938) · BMJ · doi:10.1136/bmj.2.4060.914 · Cited by 17

Chromatin unbound

Vijg, J., & Suh, Y. (2006) · Nature · doi:10.1038/440874a · Cited by 10

The subcellular distribution of the p53 tumour suppressor, and organismal ageing.

Węsierska‐Gądek, J., & Schmid, G. (2005) · PubMed · Cited by 7

Ticking Fast or Ticking Slow, Through Shc Must You Go?

Martin, F. M., & Friedman, J. S. (2004) · Science of Aging Knowledge Environment · doi:10.1126/sageke.2004.32.pe32 · Cited by 5

Cellular Mechanotransduction: From Tension to Function

Martino, F. D., Perestrelo, A. R., Vinarský, V., Pagliari, S., & Forte, G. (2018) · Frontiers in Physiology · doi:10.3389/fphys.2018.00824 · Cited by 1,028

Activation of innate immunity system during aging: NF-kB signaling is the molecular culprit of inflamm-aging

Salminen, A., Huuskonen, J., Ojala, J., Kauppinen, A., Kaarniranta, K., & Suuronen, T. (2008) · Ageing Research Reviews · doi:10.1016/j.arr.2007.09.002 · Cited by 581

Combined treatment with statins and aminobisphosphonates extends longevity in a mouse model of human premature aging

Varela, I., Pereira, S., Ugalde, A. P., Navarro, C. L., Suárez, M. F., Cau, P., Cadiñanos, J., Osorio, F. G., Foray, N., Cobo, J., et al. (2008) · Nature Medicine · doi:10.1038/nm1786 · Cited by 376

A mechanism for asymmetric segregation of age during yeast budding

Shcheprova, Z., Baldi, S., Frei, S. B., Gonnet, G. H., & Barral, Y. (2008) · Nature · doi:10.1038/nature07212 · Cited by 336

Random walk with restart on multiplex and heterogeneous biological networks

Valdeolivas, A., Tichit, L., Navarro, C. L., Perrin, S., Odelin, G., Lévy, N., Cau, P., Rémy, É., & Baudot, A. (2018) · Bioinformatics · doi:10.1093/bioinformatics/bty637 · Cited by 335

Genomic instability and DNA replication defects in progeroid syndromes

Burla, R., La Torre, M., Merigliano, C., Vernì, F., & Saggio, I. (2018) · Nucleus · doi:10.1080/19491034.2018.1476793 · Cited by 59

Molecular pathology of rare progeroid diseases

Rieckher, M., Garinis, G. A., & Schumacher, B. (2021) · Trends in Molecular Medicine · doi:10.1016/j.molmed.2021.06.011 · Cited by 50

Human progeroid syndromes, aging and cancer: new genetic and epigenetic insights into old questions

Ramírez, C. L. J., Cadiñanos, J., Varela, I., Freije, J. M. P., & López-Otín, C. (2006) · Cellular and Molecular Life Sciences · doi:10.1007/s00018-006-6349-3 · Cited by 88

Molecular Basis of Progeroid Diseases

García‐Aguirre, I., Monterrubio-Ledezma, F., Alamillo‐Iniesta, A., Castro‐Obregón, S., & Cisneros, B. (2020) · Springer eBooks · doi:10.1007/978-3-030-40955-5_10 · Cited by 2

Premature aging syndromes: From patients to mechanism

Foo, M. X. R., Ong, P. F., & Dreesen, O. (2019) · Journal of Dermatological Science · doi:10.1016/j.jdermsci.2019.10.003 · Cited by 53