{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,7]],"date-time":"2026-11-07T02:53:21Z","timestamp":1794020001887,"version":"4.8.0"},"reference-count":89,"publisher":"Pleiades Publishing Ltd","issue":"9","license":[{"start":{"date-parts":[[2018,9,1]],"date-time":"2018-09-01T00:00:00Z","timestamp":1535760000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2018,9,1]],"date-time":"2018-09-01T00:00:00Z","timestamp":1535760000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Biochemistry Moscow"],"published-print":{"date-parts":[[2018,9]]},"DOI":"10.1134\/s0006297918090043","type":"journal-article","created":{"date-parts":[[2018,9,17]],"date-time":"2018-09-17T00:09:07Z","timestamp":1537142947000},"page":"1030-1039","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":53,"title":["Molecular Pathogenesis in Huntington\u2019s Disease"],"prefix":"10.1134","volume":"83","author":[{"given":"S. N.","family":"Illarioshkin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S. A.","family":"Klyushnikov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V. A.","family":"Vigont","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu. A.","family":"Seliverstov","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E. V.","family":"Kaznacheyeva","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"137","published-online":{"date-parts":[[2018,9,17]]},"reference":[{"key":"656_CR1","doi-asserted-by":"publisher","first-page":"331","DOI":"10.2217\/nmt-2016-0008","volume":"6","author":"S. S. Baig","year":"2016","unstructured":"Baig, S. S., Strong, M., and Quarrell, O. W. (2016) The global prevalence of Huntington\u2019s disease: a systematic review and discussion, Neurodegener. Dis. Manag., 6, 331\u2013343.","journal-title":"Neurodegener. Dis. Manag."},{"key":"656_CR2","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4471-1308-9","volume-title":"Huntington\u2019s Chorea","author":"M. R. Hayden","year":"1981","unstructured":"Hayden, M. R. (1981) Huntington\u2019s Chorea, Springer-Verlag, Berlin."},{"key":"656_CR3","first-page":"1","volume":"1","author":"G. P. Bates","year":"2015","unstructured":"Bates, G. P., Dorsey, R., Gusella, J. F., Hayden, M. R., Kay, C., Leavitt, B. R., Nance, M., Ross, C. A., Scahill, R. I., Wetzel, R., Wild, E. J., and Tabrizi, S. J. (2015) Huntington\u2019s disease, Nature Rev. Dis. Primers, 1, 1\u201321.","journal-title":"Nature Rev. Dis. Primers"},{"key":"656_CR4","doi-asserted-by":"crossref","unstructured":"The Huntington\u2019s Disease Collaborative Research Group (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington\u2019s disease chromosomes, Cell, 72, 971\u2013983.","DOI":"10.1016\/0092-8674(93)90585-E"},{"key":"656_CR5","doi-asserted-by":"publisher","first-page":"339","DOI":"10.1086\/302269","volume":"64","author":"H. L. Paulson","year":"1999","unstructured":"Paulson, H. L. (1999) Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold, Am. J. Hum. Genet., 64, 339\u2013345.","journal-title":"Am. J. Hum. Genet."},{"key":"656_CR6","doi-asserted-by":"publisher","first-page":"695","DOI":"10.1136\/jmg.2007.050930","volume":"44","author":"V. C. Wheeler","year":"2007","unstructured":"Wheeler, V. C., Persichetti, F., McNeil, S. M., Mysore, J. S., Mysore, S. S., MacDonald, M. E., Myers, R. H., Gusella, J. F., Wexler, N. S., and The US-Venezuela Collaborative Research Group (2007) Factors associated with HD CAG repeat instability in Huntington\u2019s disease, J. Med. Gen., 44, 695\u2013701.","journal-title":"J. Med. Gen."},{"key":"656_CR7","doi-asserted-by":"publisher","first-page":"325","DOI":"10.1016\/B978-0-12-381328-2.00014-6","volume":"98","author":"A. Reiner","year":"2011","unstructured":"Reiner, A., Dragatsis, I., and Dietrich, P. (2011) Genetics and neuropathology of Huntington\u2019s disease, Int. Rev. Neurobiol., 98, 325\u2013372.","journal-title":"Int. Rev. Neurobiol."},{"key":"656_CR8","doi-asserted-by":"publisher","first-page":"387","DOI":"10.1038\/ng0893-387","volume":"4","author":"M. Duyao","year":"1993","unstructured":"Duyao, M., Ambrose, C., Myers, R., Novelletto, A., Persichetti, F., Frontali, M., Folstein, S., Ross, C., Franz, M., Abbott, M., Gray, J., Conneally, P., Young, A., Penney, J., Hollingsworth, Z., Shoulson, I., Lazzarini, A., Falek, A., Koroshetz, W., Sax, D., Bird, E., Vonsattel, J., Bonilla, E., Alvir, J., Bickham Conde, J., Cha, J.-H., Dure, L., Gomez, F., Ramos, M., Sanchez-Ramos, J., Snodgrass, S., de Young, M., Wexler, N., Moscowitz, C., Penchaszadeh, G., MacFarlane, H., Anderson, M., Jenkins, B., Srinidhi, J., Barnes, G., Gusella, J., and MacDonald, M. (1993) Trinucleotide repeat length instability and age of onset in Huntington\u2019s disease, Nat. Genet., 4, 387\u2013392.","journal-title":"Nat. Genet."},{"key":"656_CR9","doi-asserted-by":"publisher","first-page":"295","DOI":"10.1111\/j.1469-1809.2006.00335.x","volume":"71","author":"J. M. Andresen","year":"2006","unstructured":"Andresen, J. M., Javiar, G., Djousse, L., Roberts, S., Brocklebank, D., Cherny, S. S., The US-Venezuela Collaborative Research Group, HD MAPS Collaborative Research Group, Cardon, L. R., Gusella, J. F., MacDonald, M. E., Myers, R. H., Housman, D. E., and Wexler, N. S. (2006) The relationship between CAG repeat length and age of onset differs for Huntington\u2019s disease patients with juvenile onset or adult onset, Hum. Gen., 71, 295\u2013301.","journal-title":"Hum. Gen."},{"key":"656_CR10","first-page":"1202","volume":"60","author":"R. R. Brinkman","year":"1997","unstructured":"Brinkman, R. R., Mezei, M. M., Theilmann, J., Almqvist, E., and Hayden, M. R. (1997) The likelihood of being affected with Huntington\u2019s disease by a particular age, for a specific CAG size, Am. J. Hum. Genet., 60, 1202\u20131210.","journal-title":"Am. J. Hum. Genet."},{"key":"656_CR11","doi-asserted-by":"publisher","first-page":"159","DOI":"10.1006\/nbdi.1994.0019","volume":"1","author":"F. Persichetti","year":"1994","unstructured":"Persichetti, F., Srinidhi, J., Kanaley, L., Ge, P., Myers, R. H., D\u2019Arrigo, K., Barnes, G. T., MacDonald, M. E., Vonsattel, J. P., Gusella, J. F., and Bird, E. D. (1994) Huntington\u2019s disease CAG trinucleotide repeats in pathologically confirmed post-mortem brains, Neurobiol. Dis., 1, 159\u2013166.","journal-title":"Neurobiol. Dis."},{"key":"656_CR12","doi-asserted-by":"publisher","first-page":"630","DOI":"10.1002\/ana.410360412","volume":"36","author":"S. N. Illarioshkin","year":"1994","unstructured":"Illarioshkin, S. N., Igarashi, S., Onodera, O., Markova, E. D., Nikolskaya, N. N., Tanaka, H., Chabrashwili, T. Z., Insarova, N. G., Endo, K., Ivanova-Smolenskaya, I. A., and Tsuji, S. (1994) Trinucleotide repeat length and rate of progression of Huntington\u2019s disease, Ann. Neurol., 36, 630\u2013635.","journal-title":"Ann. Neurol."},{"key":"656_CR13","doi-asserted-by":"publisher","first-page":"527","DOI":"10.1212\/WNL.46.2.527","volume":"46","author":"J. Brandt","year":"1996","unstructured":"Brandt, J., Bylsma, F. W., Gross, R., Stine, O. C., Ranen, N., and Ross, C. A. (1996) Trinucleotide repeat length and clinical progression in Huntington\u2019s disease, Neurology, 46, 527\u2013531.","journal-title":"Neurology"},{"key":"656_CR14","doi-asserted-by":"publisher","first-page":"1016","DOI":"10.1212\/01.wnl.0000204230.16619.d9","volume":"66","author":"A. Rosenblatt","year":"2006","unstructured":"Rosenblatt, A., Liang, K. Y., Zhou, H., Abbott, M. H., Gourley, L. M., Margolis, R. L., Brandt, J., and Ross, C. A. (2006) The association of CAG repeat length with clinical progression in Huntington\u2019s disease, Neurology, 66, 1016\u20131020.","journal-title":"Neurology"},{"key":"656_CR15","doi-asserted-by":"publisher","first-page":"255","DOI":"10.1602\/neurorx.1.2.255","volume":"1","author":"R. H. Myers","year":"2004","unstructured":"Myers, R. H. (2004) Huntington\u2019s disease genetics, NeuroRx, 1, 255\u2013262.","journal-title":"NeuroRx"},{"key":"656_CR16","doi-asserted-by":"publisher","first-page":"832","DOI":"10.1016\/0959-437X(94)90067-1","volume":"4","author":"D. Tautz","year":"1994","unstructured":"Tautz, D., and Schlotterer, C. (1994) Simple sequences, Curr. Opin. Genet. Dev., 4, 832\u2013837.","journal-title":"Curr. Opin. Genet. Dev."},{"key":"656_CR17","doi-asserted-by":"publisher","first-page":"3344","DOI":"10.1002\/elps.200406069","volume":"25","author":"M. D. E.-M. Klintschar","year":"2004","unstructured":"Klintschar, M., Dauber, E.-M., Ricci, U., Cerri, N., Immel, U. D., Kleiber, M., and Mayr, W. R. (2004) Haplotype studies support slippage as the mechanism of germline mutations in short tandem repeats, Electrophoresis, 25, 3344\u20133348.","journal-title":"Electrophoresis"},{"key":"656_CR18","doi-asserted-by":"publisher","first-page":"56","DOI":"10.1038\/scientificamerican0816-56","volume":"315","author":"C. Zuccato","year":"2016","unstructured":"Zuccato, C., and Cattaneo, E. (2016) The Huntington\u2019s paradox, Sci. Am., 315, 56\u201361.","journal-title":"Sci. Am."},{"key":"656_CR19","doi-asserted-by":"publisher","first-page":"910","DOI":"10.1016\/j.neuron.2016.02.003","volume":"89","author":"F. Saudou","year":"2016","unstructured":"Saudou, F., and Humbert, S. (2016) The biology of huntingtin, Neuron, 89, 910\u2013926.","journal-title":"Neuron"},{"key":"656_CR20","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1006\/exnr.1998.6832","volume":"152","author":"J. Velier","year":"1998","unstructured":"Velier, J., Kim, M., Schwarz, C., Kim, T. W., Sapp, E., Chase, K., Aronin, N., and DiFiglia, M. (1998) Wild-type and mutant huntingtins function in vesicle trafficking in the secretory and endocytic pathways, Exp. Neurol., 152, 34\u201340.","journal-title":"Exp. Neurol."},{"key":"656_CR21","doi-asserted-by":"publisher","first-page":"66","DOI":"10.1016\/j.neuroscience.2012.05.043","volume":"251","author":"J. Nithianantharajah","year":"2013","unstructured":"Nithianantharajah, J., and Hannan, A. J. (2013) Dysregulation of synaptic proteins, dendritic spine abnormalities and pathological plasticity of synapses as experience-dependent mediators of cognitive and psychiatric symptoms in Huntington\u2019s disease, Neuroscience, 251, 66\u201374.","journal-title":"Neuroscience"},{"key":"656_CR22","doi-asserted-by":"publisher","first-page":"26","DOI":"10.1016\/j.tins.2014.09.003","volume":"38","author":"D. D. O. Martin","year":"2014","unstructured":"Martin, D. D. O., Ladha, S., Ehrnhoefer, D. E., and Hayden, M. R. (2014) Autophagy in Huntington\u2019s disease and huntingtin in autophagy, Trends Neurosci., 38, 26\u201335.","journal-title":"Trends Neurosci."},{"key":"656_CR23","doi-asserted-by":"publisher","first-page":"5355","DOI":"10.1073\/pnas.91.12.5355","volume":"91","author":"M. F. Perutz","year":"1994","unstructured":"Perutz, M. F., Johnson, T., Suzuki, M., and Finch, J. T. (1994) Glutamine repeats as polar zippers: their possible role in inherited neurologic diseases, Proc. Natl. Acad. Sci. USA, 91, 5355\u20135358.","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"656_CR24","doi-asserted-by":"publisher","first-page":"8808","DOI":"10.1074\/jbc.M109.075028","volume":"285","author":"C. Landles","year":"2010","unstructured":"Landles, C., Sathasivam, K., Weiss, A., Woodman, B., Moffitt, H., Finkbeiner, S., Sun, B., Gafni, J., Ellerby, L. M., Trottier, Y., Richards, W. G., Osmand, A., Paganetti, P., and Bates, G. P. (2010) Proteolysis of mutant huntingtin produces an exon 1 fragment that accumulates as an aggregated protein in neuronal nuclei in Huntington\u2019s disease, J. Biol. Chem., 285, 8808\u20138823.","journal-title":"J. Biol. Chem."},{"key":"656_CR25","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1136\/jmg.36.4.265","volume":"36","author":"D. C. Rubinsztein","year":"1999","unstructured":"Rubinsztein, D. C., Wyttenbach, A., and Rankin, J. (1999) Intracellular inclusions, pathological markers in diseases caused by expanded polyglutamine tracts? J. Med. Genet., 36, 265\u2013270.","journal-title":"J. Med. Genet."},{"key":"656_CR26","doi-asserted-by":"publisher","first-page":"1005","DOI":"10.1098\/rstb.1999.0452","volume":"354","author":"C. A. Ross","year":"1999","unstructured":"Ross, C. A., Wood, J. D., and Schilling, G. (1999) Polyglutamine pathogenesis, Philos. Trans. R. Soc. Lond. B Biol. Sci., 354, 1005\u20131011.","journal-title":"Philos. Trans. R. Soc. Lond. B Biol. Sci."},{"key":"656_CR27","doi-asserted-by":"publisher","first-page":"1179","DOI":"10.1016\/j.cell.2006.04.026","volume":"125","author":"R. K. Graham","year":"2006","unstructured":"Graham, R. K., Deng, Y., and Slow, E. J. (2006) Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin, Cell, 125, 1179\u20131191.","journal-title":"Cell"},{"key":"656_CR28","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1016\/S0092-8674(00)81782-1","volume":"95","author":"F. Saudou","year":"1998","unstructured":"Saudou, F., Finkbeiner, S., Devys, D., and Greenberg, M. E. (1998) Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions, Cell, 95, 55\u201366.","journal-title":"Cell"},{"key":"656_CR29","doi-asserted-by":"publisher","first-page":"41","DOI":"10.1016\/S0092-8674(00)81781-X","volume":"95","author":"I. A. Klement","year":"1998","unstructured":"Klement, I. A., Skinner, P. A., Kaytor, M. D., Yi, H., Hersch, S. M., Clark, H. B., Zoghbi, H. Y., and Orr, H. T. (1998) Ataxin-1 nuclear localisation and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice, Cell, 95, 41\u201353.","journal-title":"Cell"},{"key":"656_CR30","doi-asserted-by":"publisher","first-page":"1990","DOI":"10.1126\/science.277.5334.1990","volume":"277","author":"M. DiFiglia","year":"1997","unstructured":"DiFiglia, M., Sapp, E., Chase, K. O., Davies, S. W., Bates, G. P., Vonsattel, J. P., and Aronin, N. (1997) Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain, Science, 277, 1990\u20131993.","journal-title":"Science"},{"key":"656_CR31","doi-asserted-by":"publisher","first-page":"549","DOI":"10.1016\/S0092-8674(00)80514-0","volume":"90","author":"E. Scherzinger","year":"1997","unstructured":"Scherzinger, E., Lurz, R., Turmaine, M., Mangiarini, L., Hollenbach, B., Hasenbank, R., Bates, G. P., Davies, S. W., Lehrach, H., and Wanker, E. E. (1997) Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo, Cell, 90, 549\u2013558.","journal-title":"Cell"},{"key":"656_CR32","doi-asserted-by":"publisher","first-page":"537","DOI":"10.1016\/S0092-8674(00)80513-9","volume":"90","author":"S. W. Davies","year":"1997","unstructured":"Davies, S. W., Turmaine, M., Cozens, B. A., DiFiglia, M., Sharp, A. H., Ross, C. A., Scherzinger, E., Wanker, E. E., Mangiarini, L., and Bates, G. P. (1997) Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation, Cell, 90, 537\u2013548.","journal-title":"Cell"},{"key":"656_CR33","doi-asserted-by":"publisher","first-page":"939","DOI":"10.1016\/S0092-8674(00)81200-3","volume":"93","author":"J. M. Warrick","year":"1998","unstructured":"Warrick, J. M., Paulson, H. L., Gray-Board, G. L., Bui, Q. T., Fischbeck, K. H., Pittman, R. N., and Bonini, N. M. (1998) Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila, Cell, 93, 939\u2013949.","journal-title":"Cell"},{"key":"656_CR34","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1186\/s13024-016-0092-5","volume":"11","author":"E. D. Nekrasov","year":"2016","unstructured":"Nekrasov, E. D., Vigont, V. A., Klyushnikov, S. A., Lebedeva, O. S., Vassina, E. M., Bogomazova, A. N., Chestkov, I. V., Semashko, T. A., Kiseleva, E., Suldina, L. A., Bobrovsky, P. A., Zimina, O. A., Ryazantseva, M. A., Skopin, A. Y., Illarioshkin, S. N., Kaznacheyeva, E. V., Lagarkova, M. A., and Kiselev, S. L. (2016) Manifestation of Huntington\u2019s disease pathology in human induced pluripotent stem cell-derived neurons, Mol. Neurodegener., 11,27.","journal-title":"Mol. Neurodegener."},{"key":"656_CR35","doi-asserted-by":"publisher","first-page":"19385","DOI":"10.1074\/jbc.271.32.19385","volume":"271","author":"M. A. Kalchman","year":"1996","unstructured":"Kalchman, M. A., Graham, R. K., Xia, G., Koide, H. B., Hodgson, J. G., Graham, K. C., Goldberg, Y. P., Gietz, R. D., Pickart, C. M., and Hayden, M. R. (1996) Huntingtin is ubiquitinated and interacts with a specific ubiquitin-conjugating enzyme, J. Biol. Chem., 271, 19385\u201319394.","journal-title":"J. Biol. Chem."},{"key":"656_CR36","doi-asserted-by":"publisher","first-page":"673","DOI":"10.1093\/hmg\/8.4.673","volume":"8","author":"Y. Chai","year":"1999","unstructured":"Chai, Y., Koppenhafer, S. L., Shoesmith, S. J., Perez, M. K., and Paulson, H. L. (1999) Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3\/MJD and suppression of poly-glutamine aggregation in vitro, Hum. Mol. Genet., 8, 673\u2013682.","journal-title":"Hum. Mol. Genet."},{"key":"656_CR37","doi-asserted-by":"publisher","first-page":"4307","DOI":"10.1038\/sj.emboj.7600426","volume":"23","author":"C. I. Holmberg","year":"2004","unstructured":"Holmberg, C. I., Staniszewski, K. E., Mensah, K. N., Matouschek, A., and Morimoto, R. I. (2004) Inefficient degradation of truncated polyglutamine proteins by the proteasome, EMBO J., 23, 4307\u20134318.","journal-title":"EMBO J."},{"key":"656_CR38","doi-asserted-by":"publisher","first-page":"378","DOI":"10.1016\/j.tibs.2013.05.003","volume":"38","author":"J. Labbadia","year":"2013","unstructured":"Labbadia, J., and Morimoto, R. I. (2013) Huntington\u2019s disease: underlying molecular mechanisms and emerging concepts, Trends Biochem. Sci., 38, 378\u2013385.","journal-title":"Trends Biochem. Sci."},{"key":"656_CR39","doi-asserted-by":"publisher","first-page":"27","DOI":"10.3389\/fnmol.2016.00027","volume":"9","author":"T. Zhao","year":"2016","unstructured":"Zhao, T., Hong, Y., Li, X.-J., and Li, S.-H. (2016) Subcellular clearance and accumulation of Huntington\u2019s disease protein: a mini-review, Front. Mol. Neurosci., 9,27.","journal-title":"Front. Mol. Neurosci."},{"key":"656_CR40","doi-asserted-by":"publisher","first-page":"585","DOI":"10.1038\/ng1362","volume":"36","author":"B. Ravikumar","year":"2004","unstructured":"Ravikumar, B., Vacher, C., Berger, Z., Davies, J. E., Luo, S., Oroz, L. G., Scaravilli, F., Easton, D. F., Duden, R., O\u2019Kane, C. J., and Rubinsztein, D. C. (2004) Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington\u2019s disease, Nat. Genet., 36, 585\u2013595.","journal-title":"Nat. Genet."},{"key":"656_CR41","doi-asserted-by":"publisher","first-page":"771","DOI":"10.1038\/ng1591","volume":"37","author":"B. Ravikumar","year":"2005","unstructured":"Ravikumar, B., Acevedo-Arozena, A., Imarisio, S., Berger, Z., Vacher, C., O\u2019Kane, C. J., Brown, S. D., and Rubinsztein, D. C. (2005) Dynein mutations impair autophagic clearance of aggregate-prone proteins, Nat. Genet., 37, 771\u2013776.","journal-title":"Nat. Genet."},{"key":"656_CR42","doi-asserted-by":"publisher","first-page":"754","DOI":"10.1038\/aps.2018.11","volume":"39","author":"R. J. Harding","year":"2018","unstructured":"Harding, R. J., and Tong, Y. F. (2018) Proteostasis in Huntington\u2019s disease: disease mechanisms and therapeutic opportunities, Acta Pharmacol. Sin., 39, 754\u2013769.","journal-title":"Acta Pharmacol. Sin."},{"key":"656_CR43","doi-asserted-by":"publisher","first-page":"964","DOI":"10.1523\/JNEUROSCI.19-03-00964.1999","volume":"19","author":"M. L. H.-S. Kim","year":"1999","unstructured":"Kim, M., Lee, H.-S., LaForet, G., McIntyre, C., Martin, E. J., Chang, P., Kim, T. W., Williams, M., Reddy, P. H., Tagle, D., Boyce, F. M., Won, L., Heller, A., Aronin, N., and DiFiglia, M. (1999) Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition, J. Neurosci., 19, 964\u2013973.","journal-title":"J. Neurosci."},{"key":"656_CR44","doi-asserted-by":"publisher","first-page":"879","DOI":"10.1016\/S0896-6273(00)81035-1","volume":"24","author":"C. J. Cummings","year":"1999","unstructured":"Cummings, C. J., Reinstein, E., Sun, Y., Antalffy, B., Jiang, Y., Ciechanover, A., Orr, H. T., Beaudet, A. L., and Zoghbi, H. Y. (1999) Mutation of the E6-AP ubiquitin ligase reduces nuclear inclusion frequency while accelerating polyglutamine-induced pathology in SCA1 mice, Neuron, 24, 879\u2013892.","journal-title":"Neuron"},{"key":"656_CR45","doi-asserted-by":"publisher","first-page":"406","DOI":"10.1007\/s12035-014-8715-8","volume":"51","author":"L. M. Valor","year":"2015","unstructured":"Valor, L. M. (2015) Transcription, epigenetics and ameliorative strategies in Huntington\u2019s disease: a genome-wide perspective, Mol. Neurobiol., 51, 406\u2013423.","journal-title":"Mol. Neurobiol."},{"key":"656_CR46","volume-title":"Neurobiology of Huntington\u2019s Disease: Applications to Drug Discovery","author":"A. R. La Spada","year":"2011","unstructured":"La Spada, A. R., Weydt, P., and Pineda, V. V. (2011) Huntington\u2019s disease pathogenesis: mechanisms and path-ways, in Neurobiology of Huntington\u2019s Disease: Applications to Drug Discovery (Lo, D. C., and Hughes, R. E., eds.), Chap. 2, Taylor & Francis, Boca Raton."},{"key":"656_CR47","doi-asserted-by":"publisher","first-page":"560","DOI":"10.1016\/j.mcn.2005.05.003","volume":"30","author":"S. Y. Cong","year":"2005","unstructured":"Cong, S. Y., Pepers, B. A., Evert, B. O., Rubinsztein, D. C., Roos, R. A., van Ommen, G. J., and Dorsman, J. C. (2005) Mutant huntingtin represses CBP, but not p300, by binding and protein degradation, Mol. Cell. Neurosci., 30, 560\u2013571.","journal-title":"Mol. Cell. Neurosci."},{"key":"656_CR48","doi-asserted-by":"publisher","first-page":"16672","DOI":"10.1074\/jbc.M511648200","volume":"281","author":"Z. Qiu","year":"2006","unstructured":"Qiu, Z., Norflus, F., Singh, B., Swindell, M. K., Buzescu, R., Bejarano, M., Chopra, R., Zucker, B., Benn, C. L., DiRocco, D. P., Cha, J. H., Ferrante, R. J., and Hersch, S. M. (2006) Sp1 is up-regulated in cellular and transgenic models of Huntington\u2019s disease, and its reduction is neuroprotective, J. Biol. Chem., 281, 16672\u201316680.","journal-title":"J. Biol. Chem."},{"key":"656_CR49","doi-asserted-by":"publisher","first-page":"438","DOI":"10.1136\/jmg.2009.066399","volume":"46","author":"M. Futter","year":"2009","unstructured":"Futter, M., Diekmann, H., Schoenmakers, E., Sadiq, O., Chatterjee, K., and Rubinsztein, D. C. (2009) Wild-type but not mutant huntingtin modulates the transcriptional activity of liver X receptors, J. Med. Genet., 46, 438\u2013446.","journal-title":"J. Med. Genet."},{"key":"656_CR50","doi-asserted-by":"publisher","first-page":"294","DOI":"10.1016\/j.pneurobio.2007.01.003","volume":"81","author":"C. Zuccato","year":"2007","unstructured":"Zuccato, C., and Cattaneo, E. (2007) Role of brain-derived neurotrophic factor in Huntington\u2019s disease, Prog. Neurobiol., 81, 294\u2013330.","journal-title":"Prog. Neurobiol."},{"key":"656_CR51","doi-asserted-by":"publisher","first-page":"357","DOI":"10.1101\/gad.1177604","volume":"18","author":"D. P. Kelly","year":"2004","unstructured":"Kelly, D. P., and Scarpulla, R. C. (2004) Transcriptional regulatory circuits controlling mitochondrial biogenesis and function, Genes Dev., 18, 357\u2013368.","journal-title":"Genes Dev."},{"key":"656_CR52","doi-asserted-by":"publisher","first-page":"4181","DOI":"10.1523\/JNEUROSCI.13-10-04181.1993","volume":"13","author":"M. F. Beal","year":"1993","unstructured":"Beal, M. F., Brouillet, E., Jenkins, B. G., Ferrante, R. J., Kowall, N. W., Miller, J. M., Storey, E., Srivastava, R., Rosen, B. R., and Hyman, B. T. (1993) Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid, J. Neurosci., 13, 4181\u20134189.","journal-title":"J. Neurosci."},{"key":"656_CR53","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1002\/1531-8249(199901)45:1<25::AID-ART6>3.0.CO;2-E","volume":"45","author":"S. J. Tabrizi","year":"1999","unstructured":"Tabrizi, S. J., Cleeter, M. W., Xuereb, J., Taanman, J. W., Cooper, J. M., and Schapira, A. H. (1999) Biochemical abnormalities and excitotoxicity in Huntington\u2019s disease brain, Ann. Neurol., 45, 25\u201332.","journal-title":"Ann. Neurol."},{"key":"656_CR54","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1016\/j.freeradbiomed.2013.04.016","volume":"62","author":"A. Johri","year":"2013","unstructured":"Johri, A., Chandra, A., and Beal, M. F. (2013) PGC-1a, mitochondrial dysfunction, and Huntington\u2019s disease, Free Radic. Biol. Med., 62, 37\u201346.","journal-title":"Free Radic. Biol. Med."},{"key":"656_CR55","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1136\/jnnp.62.1.27","volume":"62","author":"L. Harms","year":"1997","unstructured":"Harms, L., Meierkord, H., Timm, G., Pfeiffer, L., and Ludolph, A. C. (1997) Decreased N-acetylaspartate\/choline ratio and increased lactate in the frontal lobe of patients with Huntington\u2019s disease: a proton magnetic resonance spectroscopy study, J. Neurol. Neurosurg. Psychiatry, 62, 27\u201330.","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"656_CR56","doi-asserted-by":"publisher","first-page":"101","DOI":"10.1016\/j.bbadis.2011.10.016","volume":"1822","author":"P. H. Reddy","year":"2012","unstructured":"Reddy, P. H., and Shirendeb, U. P. (2012) Mutant huntingtin, abnormal mitochondrial dynamics, defective axonal transport of mitochondria, and selective synaptic degeneration in Huntington\u2019s disease, Biochim. Biophys. Acta, 1822, 101\u2013110.","journal-title":"Biochim. Biophys. Acta"},{"key":"656_CR57","doi-asserted-by":"publisher","first-page":"2783","DOI":"10.1523\/JNEUROSCI.0106-08.2008","volume":"28","author":"A. L. Orr","year":"2008","unstructured":"Orr, A. L., Li, S., Wang, C. E., Li, H., Wang, J., Rong, J., Xu, X., Mastroberardino, P. G., Greenamyre, J. T., and Li, X. J. (2008) N-terminal mutant huntingtin associates with mitochondria and impairs mitochondrial trafficking, J. Neurosci., 28, 2783\u20132792.","journal-title":"J. Neurosci."},{"key":"656_CR58","doi-asserted-by":"publisher","first-page":"1939","DOI":"10.1089\/ars.2010.3779","volume":"14","author":"G. Twig","year":"2011","unstructured":"Twig, G., and Shirihai, O. S. (2011) The interplay between mitochondrial dynamics and mitophagy, Antioxid. Redox Signal., 14, 1939\u20131951.","journal-title":"Antioxid. Redox Signal."},{"key":"656_CR59","doi-asserted-by":"publisher","first-page":"567","DOI":"10.1038\/nn.2528","volume":"13","author":"M. Martinez-Vicente","year":"2010","unstructured":"Martinez-Vicente, M., Talloczy, Z., Wong, E., Tang, G., Koga, H., Kaushik, S., de Vries, R., Arias, E., Harris, S., Sulzer, D., and Cuervo, A. M. (2010) Cargo recognition failure is responsible for inefficient autophagy in Huntington\u2019s disease, Nat. Neurosci., 13, 567\u2013576.","journal-title":"Nat. Neurosci."},{"key":"656_CR60","doi-asserted-by":"publisher","first-page":"78","DOI":"10.1210\/er.2002-0012","volume":"24","author":"P. Puigserver","year":"2003","unstructured":"Puigserver, P., and Spiegelman, B. M. (2003) Peroxisome proliferator-activated receptor-gamma coactivator 1 alpha (PGC-1 alpha): transcriptional coactivator and metabolic regulator, Endocr. Rev., 24, 78\u201390.","journal-title":"Endocr. Rev."},{"key":"656_CR61","doi-asserted-by":"publisher","first-page":"121","DOI":"10.1016\/j.cell.2004.09.013","volume":"119","author":"J. Lin","year":"2004","unstructured":"Lin, J., Wu, P. H., Tarr, P. T., Lindenberg, K. S., St.-Pierre, J., Zhang, C. Y., Mootha, V. K., Jager, S., Vianna, C. R., Reznick, R. M., Cui, L., Manieri, M., Donovan, M. X., Wu, Z., Cooper, M. P., Fan, M. C., Rohas, L. M., Zavacki, A. M., Cinti, S., Shulman, G. I., Lowell, B. B., Krainc, D., and Spiegelman, B. M. (2004) Defects in adaptive energy metabolism with CNS-linked hyperactivity in PGC-1alpha null mice, Cell, 119, 121\u2013123.","journal-title":"Cell"},{"key":"656_CR62","doi-asserted-by":"publisher","first-page":"e101","DOI":"10.1371\/journal.pbio.0030101","volume":"3","author":"T. C. Leone","year":"2005","unstructured":"Leone, T. C., Lehman, J. J., and Finck, B. N. (2005) PGC-1alpha deficiency causes multi-system energy metabolic derangements: muscle dysfunction, abnormal weight control and hepatic steatosis, PLoS Biol., 3, e101.","journal-title":"PLoS Biol."},{"key":"656_CR63","doi-asserted-by":"publisher","first-page":"46","DOI":"10.1001\/archneur.62.1.46","volume":"62","author":"S. Gunawardena","year":"2005","unstructured":"Gunawardena, S., and Goldstein, L. S. (2005) Polyglutamine diseases and transport problems: deadly traffic jams on neuronal highways, Arch. Neurol., 62, 46\u201351.","journal-title":"Arch. Neurol."},{"key":"656_CR64","doi-asserted-by":"publisher","first-page":"479","DOI":"10.1016\/j.cell.2012.12.029","volume":"152","author":"D. Zala","year":"2013","unstructured":"Zala, D., Hinckelmann, M. V., Yu, H., Lyra da Cunha, M. M., Liot, G., Cordelieres, F. P., Marco, S., and Saudou, F. (2013) Vesicular glycolysis provides on-board energy for fast axonal transport, Cell, 152, 479\u2013491.","journal-title":"Cell"},{"key":"656_CR65","doi-asserted-by":"publisher","first-page":"127","DOI":"10.1016\/j.cell.2004.06.018","volume":"118","author":"L. R. Gauthier","year":"2004","unstructured":"Gauthier, L. R., Charrin, B. C., Borrell-Pages, M., Dompierre, J. P., Rangone, H., Cordelieres, F. P., De Mey, J., MacDonald, M. E., Lessmann, V., Humbert, S., and Saudou, F. (2004) Huntingtin controls neurotrophic support and survival of neurons by enhancing BDNF vesicular transport along microtubules, Cell, 118, 127\u2013138.","journal-title":"Cell"},{"key":"656_CR66","doi-asserted-by":"publisher","first-page":"8195","DOI":"10.1128\/MCB.24.18.8195-8209.2004","volume":"24","author":"E. Trushina","year":"2004","unstructured":"Trushina, E., Dyer, R. B., Badger, J. D., Ure, D., Eide, L., Tran, D. D., Vrieze, B. T., Legendre-Guillemin, V., McPherson, P. S., Mandavilli, B. S., Van Houten, B., Zeitlin, S., McNiven, M., Aebersold, R., Hayden, M., Parisi, J. E., Seeberg, E., Dragatsis, I., Doyle, K., Bender, A., Chacko, C., and McMurray, C. T. (2004) Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro, Mol. Cell. Biol., 24, 8195\u20138209.","journal-title":"Mol. Cell. Biol."},{"key":"656_CR67","doi-asserted-by":"publisher","first-page":"364","DOI":"10.1016\/j.it.2015.04.007","volume":"36","author":"A. Crotti","year":"2015","unstructured":"Crotti, A., and Glass, C. K. (2015) The choreography of neuroinflammation in Huntington\u2019s disease, Trends Immunol., 36, 364\u2013373.","journal-title":"Trends Immunol."},{"key":"656_CR68","doi-asserted-by":"publisher","first-page":"1314","DOI":"10.1126\/science.1110647","volume":"308","author":"A. Nimmerjahn","year":"2005","unstructured":"Nimmerjahn, A., Kirchhoff, F., and Helmchen, F. (2005) Resting microglial cells are highly dynamic surveillants of brain parenchyma in vivo, Science, 308, 1314\u20131318.","journal-title":"Science"},{"key":"656_CR69","doi-asserted-by":"publisher","first-page":"206","DOI":"10.1002\/glia.10144","volume":"40","author":"R. B. Banati","year":"2002","unstructured":"Banati, R. B. (2002) Visualizing microglial activation in vivo, Glia, 40, 206\u2013217.","journal-title":"Glia"},{"key":"656_CR70","doi-asserted-by":"publisher","first-page":"161","DOI":"10.1093\/jnen\/60.2.161","volume":"60","author":"E. Sapp","year":"2001","unstructured":"Sapp, E., Kegel, K. B., Aronin, N., Hashikawa, T., Uchiyama, Y., Tohyama, K., Bhide, P. G., Vonsattel, J. P., and DiFiglia, M. (2001) Early and progressive accumulation of reactive microglia in the Huntington\u2019s disease brain, J. Neuropathol. Exp. Neurol., 60, 161\u2013172.","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"656_CR71","doi-asserted-by":"publisher","first-page":"1759","DOI":"10.1093\/brain\/awm044","volume":"130","author":"Y. F. Tai","year":"2007","unstructured":"Tai, Y. F., Pavese, N., Gerhard, A., Tabrizi, S. J., Barker, R. A., Brooks, D. J., and Piccini, P. (2007) Microglial activation in presymptomatic Huntington\u2019s disease gene carriers, Brain, 130, 1759\u20131766.","journal-title":"Brain"},{"key":"656_CR72","doi-asserted-by":"publisher","first-page":"513","DOI":"10.1038\/nn.3668","volume":"4","author":"A. Crotti","year":"2014","unstructured":"Crotti, A., Benner, C., Kerman, B. E., Lagier-Tourenne, C., Zuccato, C., Cattaneo, E., Gage, F. H., Cleveland, D. W., and Glass, C. K. (2014) Mutant Huntingtin promotes autonomous microglia activation via myeloid lineage-determining factors, Nat. Neurosci., 4, 513\u2013521.","journal-title":"Nat. Neurosci."},{"key":"656_CR73","doi-asserted-by":"publisher","first-page":"217","DOI":"10.3233\/JHD-160205","volume":"5","author":"A. Kumar","year":"2016","unstructured":"Kumar, A., and Ratan, R. R. (2016) Oxidative stress and Huntington\u2019s disease: the good, the bad, and the ugly, J. Huntington\u2019s Dis., 5, 217\u2013237.","journal-title":"J. Huntington\u2019s Dis."},{"key":"656_CR74","doi-asserted-by":"publisher","first-page":"44512","DOI":"10.1074\/jbc.M111.307587","volume":"286","author":"S. Hands","year":"2011","unstructured":"Hands, S., Sajjad, M. U., Newton, M. J., and Wyttenbach, A. (2011) In vitro and in vivo aggregation of a fragment of huntingtin protein directly causes free radical production, J. Biol. Chem., 286, 44512\u201344520.","journal-title":"J. Biol. Chem."},{"key":"656_CR75","doi-asserted-by":"publisher","first-page":"57","DOI":"10.1038\/nrn2038","volume":"8","author":"M. L. Block","year":"2007","unstructured":"Block, M. L., Zecca, L., and Hong, J. S. (2007) Microglia-mediated neurotoxicity: uncovering the molecular mechanisms, Nat. Rev. Neurosci., 8, 57\u201369.","journal-title":"Nat. Rev. Neurosci."},{"key":"656_CR76","doi-asserted-by":"publisher","first-page":"5428","DOI":"10.1111\/j.1742-4658.2006.05537.x","volume":"273","author":"W. J. Firdaus","year":"2006","unstructured":"Firdaus, W. J., Wyttenbach, A., Giuliano, P., Kretz-Remy, C., Currie, R. W., and Arrigo, A. P. (2006) Huntingtin inclusion bodies are iron-dependent centers of oxidative events, FEBS J., 273, 5428\u20135441.","journal-title":"FEBS J."},{"key":"656_CR77","doi-asserted-by":"publisher","first-page":"181","DOI":"10.1016\/S0896-6273(00)80764-3","volume":"23","author":"J. G. Hodgson","year":"1999","unstructured":"Hodgson, J. G., Agopyan, N., Gutekunst, C.-A., Leavitt, B. R., LePiane, F., Singaraja, R., Smith, D. J., Bissada, N., McCutcheon, K., Nasir, J., Jamot, L., Li, X. J., Stevens, M. E., Rosemond, E., Roder, J. C., Phillips, A. G., Rubin, E. M., Hersch, S. M., and Hayden, M. R. (1999) A YAC mouse model for Huntington\u2019s disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration, Neuron, 23, 181\u2013192.","journal-title":"Neuron"},{"key":"656_CR78","doi-asserted-by":"publisher","first-page":"2211","DOI":"10.1016\/j.celrep.2018.04.052","volume":"23","author":"N. H. Skotte","year":"2018","unstructured":"Skotte, N. H., Andersen, J. V., Santos, A., Aldana, B. I., Willert, C. W., Norremolle, A., Waagepetersen, H. S., and Nielsen, M. L. (2018) Integrative characterization of the R6\/2 mouse model of Huntington\u2019s disease reveals dysfunctional astrocyte metabolism, Cell Rep., 23, 2211\u20132224.","journal-title":"Cell Rep."},{"key":"656_CR79","doi-asserted-by":"publisher","first-page":"713","DOI":"10.1093\/hmg\/ddi067","volume":"14","author":"J. C. Lievens","year":"2005","unstructured":"Lievens, J. C., Rival, T., Iche, M., Chneiweiss, H., and Birman, S. (2005) Expanded polyglutamine peptides disrupt EGF receptor signaling and glutamate transporter expression in Drosophila, Hum. Mol. Genet., 14, 713\u2013724.","journal-title":"Hum. Mol. Genet."},{"key":"656_CR80","doi-asserted-by":"publisher","first-page":"7727","DOI":"10.1523\/JNEUROSCI.1197-04.2004","volume":"24","author":"J. M. Canals","year":"2004","unstructured":"Canals, J. M., Pineda, J. R., Torres-Peraza, J. F., Bosch, M., Martin-Ibanez, R., Munoz, M. T., Mengod, G., Ernfors, P., and Alberch, J. (2004) Brain-derived neurotrophic factor regulates the onset and severity of motor dysfunction associated with enkephalinergic neuronal degeneration in Huntington\u2019s disease, J. Neurosci., 24, 7727\u20137739.","journal-title":"J. Neurosci."},{"key":"656_CR81","doi-asserted-by":"publisher","first-page":"11758","DOI":"10.1523\/JNEUROSCI.2461-07.2007","volume":"27","author":"A. D. Strand","year":"2007","unstructured":"Strand, A. D., Baquet, Z. C., Aragaki, A. K., Holmans, P., Yang, L., Cleren, C., Beal, M. F., Jones, L., Kooperberg, C., Olson, J. M., and Jones, K. R. (2007) Expression profiling of Huntington\u2019s disease models suggests that brain-derived neurotrophic factor depletion plays a major role in striatal degeneration, J. Neurosci., 27, 11758\u201311768.","journal-title":"J. Neurosci."},{"key":"656_CR82","doi-asserted-by":"publisher","first-page":"623","DOI":"10.1016\/S0896-6273(00)80716-3","volume":"22","author":"I. X. C.-J. Sanchez","year":"1999","unstructured":"Sanchez, I., Xu, C.-J., Juo, P., Kakizaka, A., Blenis, J., and Yuan, J. (1999) Caspase-8 is required for cell death induced by expanded polyglutamine repeats, Neuron, 22, 623\u2013633.","journal-title":"Neuron"},{"key":"656_CR83","doi-asserted-by":"publisher","first-page":"300","DOI":"10.1038\/81593","volume":"26","author":"I. Dragatsis","year":"2000","unstructured":"Dragatsis, I., Levine, M. S., and Zeitlin, S. (2000) Inactivation of Hdh in the brain and testis results in progressive neurodegeneration and sterility in mice, Nat. Genet., 26, 300\u2013306.","journal-title":"Nat. Genet."},{"key":"656_CR84","doi-asserted-by":"publisher","first-page":"1379","DOI":"10.1093\/hmg\/ddi147","volume":"14","author":"J. M. Van Raamsdonk","year":"2005","unstructured":"Van Raamsdonk, J. M., Pearson, J., Rogers, D. A., Bissada, N., Vogl, A. W., Hayden, M. R., and Leavitt, B. R. (2005) Loss of wild-type huntingtin influences motor dysfunction and survival in the YAC128 mouse model of Huntington\u2019s disease, Hum. Mol. Genet., 14, 1379\u20131392.","journal-title":"Hum. Mol. Genet."},{"key":"656_CR85","doi-asserted-by":"publisher","first-page":"24713","DOI":"10.1074\/jbc.M103501200","volume":"276","author":"Y. Sun","year":"2001","unstructured":"Sun, Y., Savanenin, A., Reddy, P. H., and Liu, Y. F. (2001) Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95, J. Biol. Chem., 276, 24713\u201324718.","journal-title":"J. Biol. Chem."},{"key":"656_CR86","doi-asserted-by":"publisher","first-page":"17","DOI":"10.3389\/fnhum.2017.00017","volume":"11","author":"L. Francelle","year":"2017","unstructured":"Francelle, L., Lotz, C., Outeiro, T., Brouillet, E., and Merienne, K. (2017) Contribution of neuroepigenetics to Huntington\u2019s disease, Front. Hum. Neurosci., 11,17.","journal-title":"Front. Hum. Neurosci."},{"key":"656_CR87","doi-asserted-by":"publisher","first-page":"1114","DOI":"10.1212\/WNL.0000000000003725","volume":"88","author":"G. Coarelli","year":"2017","unstructured":"Coarelli, G., Diallo, A., Thion, M. S., Rinaldi, D., Calvas, F., Boukbiza, O. L., Tataru, A., Charles, P., Tranchant, C., Marelli, C., Ewenczyk, C., Tchikviladze, M., Monin, M. L., Carlander, B., Anheim, M., Brice, A., Mochel, F., Tezenas du Montcel, S., Humbert, S., and Durr, A. (2017) Low cancer prevalence in polyglutamine expansion diseases, Neurology, 88, 1114\u20131119.","journal-title":"Neurology"},{"key":"656_CR88","doi-asserted-by":"publisher","first-page":"1342","DOI":"10.1002\/(SICI)1097-0142(19991001)86:7<1342::AID-CNCR33>3.0.CO;2-3","volume":"86","author":"S. A. Sorensen","year":"1999","unstructured":"Sorensen, S. A., Fenger, K., and Olsen, J. H. (1999) Significantly lower incidence of cancer among patients with Huntington\u2019s disease: an apoptotic effect of an expanded polyglutamine tract? Cancer, 86, 1342\u20131346.","journal-title":"Cancer"},{"key":"656_CR89","doi-asserted-by":"publisher","first-page":"18","DOI":"10.1007\/s11910-017-0711-8","volume":"17","author":"T. A. Mestre","year":"2017","unstructured":"Mestre, T. A., and Sampaio, C. (2017) Huntington\u2019s disease: linking pathogenesis to the development of experimental therapeutics, Curr. Neurol. Neurosci. Rep., 17, 18.","journal-title":"Curr. Neurol. Neurosci. Rep."}],"container-title":["Biochemistry (Moscow)"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1134\/S0006297918090043.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1134\/S0006297918090043","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1134\/S0006297918090043.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,16]],"date-time":"2026-03-16T00:10:05Z","timestamp":1773619805000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1134\/S0006297918090043"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,9]]},"references-count":89,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2018,9]]}},"alternative-id":["656"],"URL":"https:\/\/doi.org\/10.1134\/s0006297918090043","relation":{},"ISSN":["0006-2979","1608-3040"],"issn-type":[{"value":"0006-2979","type":"print"},{"value":"1608-3040","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,9]]},"assertion":[{"value":"21 May 2018","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"31 May 2018","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 September 2018","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}]}}