Trehalose alleviates polyglutamine-mediated pathology in a mouse model of Huntington disease

Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice

Mangiarini, L., Sathasivam, K., Seller, M., Cozens, B., Harper, A., Hetherington, C., Lawton, M., Trottier, Y., Lehrach, H., Davies, S. W., et al. (1996) · Cell · doi:10.1016/s0092-8674(00)81369-0 · Cited by 3,073

Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain

DiFiglia, M., Sapp, E., Chase, K. O., Davies, S. W., Bates, G. P., Vonsattel, J. P., & Aronin, N. (1997) · Science · doi:10.1126/science.277.5334.1990 · Cited by 2,439

Formation of Neuronal Intranuclear Inclusions Underlies the Neurological Dysfunction in Mice Transgenic for the HD Mutation

Davies, S. W., Turmaine, M., Cozens, B. A., DiFiglia, M., Sharp, A. H., Ross, C. A., Scherzinger, E., Wanker, E. E., Mangiarini, L., & Bates, G. P. (1997) · Cell · doi:10.1016/s0092-8674(00)80513-9 · Cited by 2,181

Glutamine Repeats and Neurodegeneration

Zoghbi, H. Y., & Orr, H. T. (2000) · Annual Review of Neuroscience · doi:10.1146/annurev.neuro.23.1.217 · Cited by 1,309

Huntingtin-Encoded Polyglutamine Expansions Form Amyloid-like Protein Aggregates In Vitro and In Vivo

Scherzinger, E., Lurz, R., Turmaine, M., Mangiarini, L., Hollenbach, B., Hasenbank, R., Bates, G. P., Davies, S. W., Lehrach, H., & Wanker, E. E. (1997) · Cell · doi:10.1016/s0092-8674(00)80514-0 · Cited by 1,291

Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease

Chen, M., Ona, V. O., Li, M., Ferrante, R. J., Fink, K. B., Zhu, S., Bian, J., Guo, L., Farrell, L. A., Hersch, S. M., et al. (2000) · Nature Medicine · doi:10.1038/77528 · Cited by 1,051

Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease

Hockly, E., Richon, V. M., Woodman, B., Smith, D. L., Zhou, X., Rosa, E., Sathasivam, K., Ghazi-Noori, S., Mahal, A., Lowden, P. A. S., et al. (2003) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0437870100 · Cited by 845

Chaperone suppression of aggregation and altered subcellular proteasome localization imply protein misfolding in SCA1

Cummings, C. J., Mancini, M. A., Antalffy, B., DeFranco, D. B., Orr, H. T., & Zoghbi, H. Y. (1998) · Nature Genetics · doi:10.1038/502 · Cited by 744

Multiple Effects of Trehalose on Protein Folding In Vitro and In Vivo

Singer, M. A., & Lindquist, S. (1998) · Molecular Cell · doi:10.1016/s1097-2765(00)80064-7 · Cited by 698

Expanded Polyglutamine Protein Forms Nuclear Inclusions and Causes Neural Degeneration in Drosophila

Warrick, J. M., Paulson, H. L., Gray-Board, G. L., Bui, Q. T., Fischbeck, K. H., Pittman, R. N., & Bonini, N. M. (1998) · Cell · doi:10.1016/s0092-8674(00)81200-3 · Cited by 668

Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease

Ona, V. O., Li, M., Vonsattel, J. P. G., Andrews, L. J., Khan, S. Q., Chung, W. M., Frey, A. S., Menon, A. S., Li, X.-J., Stieg, P. E., et al. (1999) · Nature · doi:10.1038/20446 · Cited by 638

Caspase Cleavage of Gene Products Associated with Triplet Expansion Disorders Generates Truncated Fragments Containing the Polyglutamine Tract

Wellington, C. L., Ellerby, L. M., Hackam, A. S., Margolis, R. L., Trifiro, M. A., Singaraja, R., McCutcheon, K., Salvesen, G. S., Propp, S. S., Bromm, M., et al. (1998) · Journal of Biological Chemistry · doi:10.1074/jbc.273.15.9158 · Cited by 562

Epidermal immunization by a needle-free powder delivery technology: Immunogenicity of influenza vaccine and protection in mice

Chen, D., Endres, R. L., Erickson, C. A., Weis, K. F., McGregor, M. W., Kawaoka, Y., & Payne, L. G. (2000) · Nature Medicine · doi:10.1038/80538 · Cited by 547

Huntingtin aggregation and toxicity in Huntington's disease

Bates, G. (2003) · The Lancet · doi:10.1016/s0140-6736(03)13304-1 · Cited by 525

Neuroprotective Effects of Creatine in a Transgenic Mouse Model of Huntington's Disease

Ferrante, R. J., Andreassen, O. A., Jenkins, B. G., Dedeoglu, A., Kuemmerle, S., Kubilus, J. K., Kaddurah-Daouk, R., Hersch, S. M., & Beal, M. F. (2000) · The Journal of Neuroscience · doi:10.1523/jneurosci.20-12-04389.2000 · Cited by 522

Pivotal role of oligomerization in expanded polyglutamine neurodegenerative disorders

Sánchez, I., Mahlke, C., & Yuan, J. (2003) · Nature · doi:10.1038/nature01301 · Cited by 503

Polyglutamine length-dependent interaction of Hsp40 and Hsp70 family chaperones with truncated N-terminal huntingtin: their role in suppression of aggregation and cellular toxicity

Jana, N. R. (2000) · Human Molecular Genetics · doi:10.1093/hmg/9.13.2009 · Cited by 432

Trehalose synthesis is induced upon exposure of Escherichia coli to cold and is essential for viability at low temperatures

Kandror, O., DeLeon, A., & Goldberg, A. L. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.142314099 · Cited by 421

Expanded polyglutamine stretches interact with TAFII130, interfering with CREB-dependent transcription

Shimohata, T., Nakajima, T., Yamada, M., Uchida, C., Onodera, O., Naruse, S., Kimura, T., Koide, R., Nozaki, K., Sano, Y., et al. (2000) · Nature Genetics · doi:10.1038/79139 · Cited by 393

Trehalose expression confers desiccation tolerance on human cells

Guo, N., Puhlev, I., Brown, D. R., Mansbridge, J., & Levine, F. (2000) · Nature Biotechnology · doi:10.1038/72616 · Cited by 388

Aggregated polyglutamine peptides delivered to nuclei are toxic to mammalian cells

Yang, W. (2002) · Human Molecular Genetics · doi:10.1093/hmg/11.23.2905 · Cited by 371

Huntingtin Spheroids and Protofibrils as Precursors in Polyglutamine Fibrilization

Poirier, M. A., Li, H., Macosko, J., Cai, S., Amzel, M., & Ross, C. A. (2002) · Journal of Biological Chemistry · doi:10.1074/jbc.m205809200 · Cited by 367

Recruitment and the Role of Nuclear Localization in Polyglutamine-mediated Aggregation

Perez, M. K., Paulson, H. L., Pendse, S. J., Saionz, S. J., Bonini, N. M., & Pittman, R. N. (1998) · The Journal of Cell Biology · doi:10.1083/jcb.143.6.1457 · Cited by 314

Formation of Polyglutamine Inclusions in Non-CNS Tissue

Sathasivam, K., Hobbs, C., Turmaine, M., Mangiarini, L., Mahal, A., Bertaux, F., Wanker, E. E., Doherty, P., Davies, S. W., & Bates, G. P. (1999) · Human Molecular Genetics · doi:10.1093/hmg/8.5.813 · Cited by 289

Inhibition of huntingtin fibrillogenesis by specific antibodies and small molecules: Implications for Huntington's disease therapy

Heiser, V., Scherzinger, E., Boeddrich, A., Nordhoff, E., Lurz, R., Schugardt, N., Lehrach, H., & Wanker, E. E. (2000) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.110138997 · Cited by 262

Pathogenesis of polyglutamine disorders: aggregation revisited

Michalik, A., & Van Broeckhoven, C. (2003) · Human Molecular Genetics · doi:10.1093/hmg/ddg295 · Cited by 232

Mice transgenic for an expanded CAG repeat in the Huntington's disease gene develop diabetes.

Hurlbert, M. S., Zhou, W., Wasmeier, C., Kaddis, F. G., Hutton, J. C., & Freed, C. R. (1999) · Diabetes · doi:10.2337/diabetes.48.3.649 · Cited by 218

Identification of benzothiazoles as potential polyglutamine aggregation inhibitors of Huntington's disease by using an automated filter retardation assay

Heiser, V., Engemann, S., Bröcker, W., Dunkel, I., Boeddrich, A., Waelter, S., Nordhoff, E., Lurz, R., Schugardt, N., Rautenberg, S., et al. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.182426599 · Cited by 205

A cellular model that recapitulates major pathogenic steps of Huntington's disease

Lunkes, A. (1998) · Human Molecular Genetics · doi:10.1093/hmg/7.9.1355 · Cited by 189

Inhibition of Polyglutamine Protein Aggregation and Cell Death by Novel Peptides Identified by Phage Display Screening

Nagai, Y., Tucker, T., Ren, H., Kenan, D. J., Henderson, B. S., Keene, J. D., Strittmatter, W. J., & Burke, J. R. (2000) · Journal of Biological Chemistry · doi:10.1074/jbc.275.14.10437 · Cited by 185

A bivalent Huntingtin binding peptide suppresses polyglutamine aggregation and pathogenesis in Drosophila

Kazantsev, A., Walker, H. A., Slepko, N., Bear, J. E., Preisinger, E., Steffan, J. S., Zhu, Y.-Z., Gertler, F. B., Housman, D. E., Marsh, J. L., et al. (2002) · Nature Genetics · doi:10.1038/ng864 · Cited by 172

Diabetes mellitus in Huntington disease

Farrer, L. A. (1985) · Clinical Genetics · doi:10.1111/j.1399-0004.1985.tb00185.x · Cited by 170

Polyglutamine fibrillogenesis: The pathway unfolds

Ross, C. A., Poirier, M. A., Wanker, E. E., & Amzel, L. M. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0237018100 · Cited by 138

Increased motoneuron survival and improved neuromuscular function in transgenic ALS mice after intraspinal injection of an adeno-associated virus encoding Bcl-2

Azzouz, M. (2000) · Human Molecular Genetics · doi:10.1093/hmg/9.5.803 · Cited by 136

Intra- and Intermolecular β-Pleated Sheet Formation in Glutamine-repeat Inserted Myoglobin as a Model for Polyglutamine Diseases

Tanaka, M., Morishima, I., Akagi, T., Hashikawa, T., & Nukina, N. (2001) · Journal of Biological Chemistry · doi:10.1074/jbc.m107502200 · Cited by 108

Caspase activation during apoptotic cell death induced by expanded polyglutamine in N2a cells

Wang, G., Mitsui, K., Kotliarova, S. E., Yamashita, A., Nagao, Y., Tokuhiro, S., Iwatsubo, T., Kanazawa, I., & Nukina, N. (1999) · Neuroreport · doi:10.1097/00001756-199908200-00001 · Cited by 98

Polyglutamine and CBP: Fatal attraction?

McCampbell, A., & Fischbeck, K. H. (2001) · Nature Medicine · doi:10.1038/87842 · Cited by 83

Therapeutic opportunities in polyglutamine disease

Hughes, R. E., & Olson, J. M. (2001) · Nature Medicine · doi:10.1038/86486 · Cited by 76

Protein aggregation and neurodegenerative disease

Ross, C. A., & Poirier, M. A. (2004) · Nature Medicine · doi:10.1038/nm1066 · Cited by 3,454

Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death

Arrasate, M., Mitra, S., Schweitzer, E. S., Segal, M. R., & Finkbeiner, S. (2004) · Nature · doi:10.1038/nature02998 · Cited by 1,979

Autophagy and Neurodegeneration: Pathogenic Mechanisms and Therapeutic Opportunities

Menzies, F. M., Fleming, A., Caricasole, A., Bento, C. F., Andrews, S. P., Ashkenazi, A., Füllgrabe, J., Jackson, A., Jimenez Sanchez, M., Karabiyik, C., et al. (2017) · Neuron · doi:10.1016/j.neuron.2017.01.022 · Cited by 1,134

Trehalose, a Novel mTOR-independent Autophagy Enhancer, Accelerates the Clearance of Mutant Huntingtin and α-Synuclein

Sarkar, S., Davies, J. E., Huang, Z., Tunnacliffe, A., & Rubinsztein, D. C. (2007) · Journal of Biological Chemistry · doi:10.1074/jbc.m609532200 · Cited by 1,142

Degradation of misfolded proteins in neurodegenerative diseases: Therapeutic targets and strategies

Ciechanover, A., & Kwon, Y. T. (2015) · Experimental & Molecular Medicine · doi:10.1038/emm.2014.117 · Cited by 898