{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T20:04:54Z","timestamp":1791317094199,"version":"4.3.1"},"reference-count":158,"publisher":"Springer Science and Business Media LLC","issue":"8","license":[{"start":{"date-parts":[[2012,8,30]],"date-time":"2012-08-30T00:00:00Z","timestamp":1346284800000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Cell Death Dis"],"DOI":"10.1038\/cddis.2012.121","type":"journal-article","created":{"date-parts":[[2012,8,30]],"date-time":"2012-08-30T10:07:57Z","timestamp":1346321277000},"page":"e382-e382","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":97,"title":["An in vitro perspective on the molecular mechanisms underlying mutant huntingtin protein toxicity"],"prefix":"10.1038","volume":"3","author":[{"given":"G","family":"Cisbani","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"F","family":"Cicchetti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2012,8,30]]},"reference":[{"key":"BFcddis2012121_CR1","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1097\/00005053-199303000-00008","volume":"181","author":"JC Rothlind","year":"1993","unstructured":"Rothlind JC, Bylsma FW, Peyser C, Folstein SE, Brandt J . Cognitive and motor correlates of everyday functioning in early Huntington\u2019s disease. J Nerv Ment Dis 1993; 181: 194\u2013199.","journal-title":"J Nerv Ment Dis"},{"issue":"Suppl 3","key":"BFcddis2012121_CR2","doi-asserted-by":"crossref","first-page":"S135","DOI":"10.1016\/S1353-8020(09)70800-4","volume":"15","author":"CA Ross","year":"2009","unstructured":"Ross CA, Shoulson I . Huntington disease: pathogenesis, biomarkers, and approaches to experimental therapeutics. Parkinsonism Relat Disord 2009; 15 (Suppl 3): S135\u2013S138.","journal-title":"Parkinsonism Relat Disord"},{"key":"BFcddis2012121_CR3","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1097\/00005072-198511000-00003","volume":"44","author":"JP Vonsattel","year":"1985","unstructured":"Vonsattel JP, Myers RH, Stevens TJ, Ferrante RJ, Bird ED, Richardson EP . Neuropathological classification of Huntington\u2019s disease. J Neuropathol Exp Neurol 1985; 44: 559\u2013577.","journal-title":"J Neuropathol Exp Neurol"},{"key":"BFcddis2012121_CR4","doi-asserted-by":"crossref","first-page":"919","DOI":"10.1038\/nrn1806","volume":"6","author":"E Cattaneo","year":"2005","unstructured":"Cattaneo E, Zuccato C, Tartari M . Normal huntingtin function: an alternative approach to Huntington\u2019s disease. Nat Rev Neurosci 2005; 6: 919\u2013930.","journal-title":"Nat Rev Neurosci"},{"key":"BFcddis2012121_CR5","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/j.tcb.2009.01.005","volume":"19","author":"JP Caviston","year":"2009","unstructured":"Caviston JP, Holzbaur EL . Huntingtin as an essential integrator of intracellular vesicular trafficking. Trends Cell Biol 2009; 19: 147\u2013155.","journal-title":"Trends Cell Biol"},{"key":"BFcddis2012121_CR6","doi-asserted-by":"crossref","first-page":"1901","DOI":"10.1007\/s00018-005-5084-5","volume":"62","author":"R Smith","year":"2005","unstructured":"Smith R, Brundin P, Li JY . Synaptic dysfunction in Huntington\u2019s disease: a new perspective. Cell Mol Life Sci 2005; 62: 1901\u20131912.","journal-title":"Cell Mol Life Sci"},{"key":"BFcddis2012121_CR7","doi-asserted-by":"crossref","first-page":"6763","DOI":"10.1073\/pnas.100110097","volume":"97","author":"JS Steffan","year":"2000","unstructured":"Steffan JS, Kazantsev A, Spasic-Boskovic O, Greenwald M, Zhu YZ, Gohler H et al. The Huntington\u2019s disease protein interacts with p53 and CREB-binding protein and represses transcription. Proc Natl Acad Sci USA 2000; 97: 6763\u20136768.","journal-title":"Proc Natl Acad Sci USA"},{"key":"BFcddis2012121_CR8","doi-asserted-by":"crossref","first-page":"182","DOI":"10.1016\/S0166-2236(00)01721-5","volume":"24","author":"E Cattaneo","year":"2001","unstructured":"Cattaneo E, Rigamonti D, Goffredo D, Zuccato C, Squitieri F, Sipione S . Loss of normal huntingtin function: new developments in Huntington\u2019s disease research. Trends Neurosci 2001; 24: 182\u2013188.","journal-title":"Trends Neurosci"},{"key":"BFcddis2012121_CR9","doi-asserted-by":"crossref","first-page":"1121","DOI":"10.1111\/j.1471-4159.2005.03605.x","volume":"96","author":"BR Leavitt","year":"2006","unstructured":"Leavitt BR, van Raamsdonk JM, Shehadeh J, Fernandes H, Murphy Z, Graham RK et al. Wild-type huntingtin protects neurons from excitotoxicity. J Neurochem 2006; 96: 1121\u20131129.","journal-title":"J Neurochem"},{"key":"BFcddis2012121_CR10","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1046\/j.1471-4159.2003.01980.x","volume":"87","author":"Y Zhang","year":"2003","unstructured":"Zhang Y, Li M, Drozda M, Chen M, Ren S, Mejia Sanchez RO et al. Depletion of wild-type huntingtin in mouse models of neurologic diseases. J Neurochem 2003; 87: 101\u2013106.","journal-title":"J Neurochem"},{"key":"BFcddis2012121_CR11","first-page":"1202","volume":"60","author":"RR Brinkman","year":"1997","unstructured":"Brinkman RR, Mezei MM, Theilmann J, Almqvist E, Hayden MR . The likelihood of being affected with Huntington disease by a particular age, for a specific CAG size. Am J Hum Genet 1997; 60: 1202\u20131210.","journal-title":"Am J Hum Genet"},{"key":"BFcddis2012121_CR12","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1002\/ajmg.a.20190","volume":"119A","author":"L Djousse","year":"2003","unstructured":"Djousse L, Knowlton B, Hayden M, Almqvist EW, Brinkman R, Ross C et al. Interaction of normal and expanded CAG repeat sizes influences age at onset of Huntington disease. Am J Med Genet A 2003; 119A: 279\u2013282.","journal-title":"Am J Med Genet A"},{"key":"BFcddis2012121_CR13","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1016\/S0092-8674(00)81369-0","volume":"87","author":"L Mangiarini","year":"1996","unstructured":"Mangiarini L, Sathasivam K, Seller M, Cozens B, Harper A, Hetherington C et al. Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell 1996; 87: 493\u2013506.","journal-title":"Cell"},{"key":"BFcddis2012121_CR14","doi-asserted-by":"crossref","first-page":"1990","DOI":"10.1126\/science.277.5334.1990","volume":"277","author":"M DiFiglia","year":"1997","unstructured":"DiFiglia M, Sapp E, Chase KO, Davies SW, Bates GP, Vonsattel JP et al. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain. Science 1997; 277: 1990\u20131993.","journal-title":"Science"},{"key":"BFcddis2012121_CR15","doi-asserted-by":"crossref","first-page":"979","DOI":"10.1111\/j.1750-3639.1997.tb00897.x","volume":"7","author":"CL Wellington","year":"1997","unstructured":"Wellington CL, Brinkman RR, O\u2019Kusky JR, Hayden MR . Toward understanding the molecular pathology of Huntington\u2019s disease. Brain Pathol 1997; 7: 979\u20131002.","journal-title":"Brain Pathol"},{"key":"BFcddis2012121_CR16","doi-asserted-by":"crossref","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 et al. Huntingtin-encoded polyglutamine expansions form amyloid-like protein aggregates in vitro and in vivo. Cell 1997; 90: 549\u2013558.","journal-title":"Cell"},{"key":"BFcddis2012121_CR17","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1023\/B:SCAM.0000007124.19463.e5","volume":"24","author":"CC Huang","year":"1998","unstructured":"Huang CC, Faber PW, Persichetti F, Mittal V, Vonsattel JP, MacDonald ME et al. Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins. Somat Cell Mol Genet 1998; 24: 217\u2013233.","journal-title":"Somat Cell Mol Genet"},{"key":"BFcddis2012121_CR18","doi-asserted-by":"crossref","first-page":"34272","DOI":"10.1074\/jbc.M109.049809","volume":"284","author":"WF Xue","year":"2009","unstructured":"Xue WF, Hellewell AL, Gosal WS, Homans SW, Hewitt EW, Radford SE . Fibril fragmentation enhances amyloid cytotoxicity. J Biol Chem 2009; 284: 34272\u201334282.","journal-title":"J Biol Chem"},{"key":"BFcddis2012121_CR19","doi-asserted-by":"crossref","first-page":"32046","DOI":"10.1074\/jbc.M201750200","volume":"277","author":"KN Dahlgren","year":"2002","unstructured":"Dahlgren KN, Manelli AM, Stine WB, Baker LK, Krafft GA, LaDu MJ . Oligomeric and fibrillar species of amyloid-beta peptides differentially affect neuronal viability. J Biol Chem 2002; 277: 32046\u201332053.","journal-title":"J Biol Chem"},{"key":"BFcddis2012121_CR20","doi-asserted-by":"crossref","first-page":"1101","DOI":"10.1007\/s00726-009-0339-3","volume":"38","author":"G Cenini","year":"2010","unstructured":"Cenini G, Cecchi C, Pensalfini A, Bonini SA, Ferrari-Toninelli G, Liguri G et al. Generation of reactive oxygen species by beta amyloid fibrils and oligomers involves different intra\/extracellular pathways. Amino Acids 2010; 38: 1101\u20131106.","journal-title":"Amino Acids"},{"key":"BFcddis2012121_CR21","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1016\/S0168-9525(03)00074-X","volume":"19","author":"KL Sugars","year":"2003","unstructured":"Sugars KL, Rubinsztein DC . Transcriptional abnormalities in Huntington disease. Trends Genet 2003; 19: 233\u2013238.","journal-title":"Trends Genet"},{"key":"BFcddis2012121_CR22","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1016\/j.expneurol.2009.05.012","volume":"219","author":"J Sassone","year":"2009","unstructured":"Sassone J, Colciago C, Cislaghi G, Silani V, Ciammola A . Huntington\u2019s disease: the current state of research with peripheral tissues. Exp Neurol 2009; 219: 385\u2013397.","journal-title":"Exp Neurol"},{"key":"BFcddis2012121_CR23","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1186\/gb-2005-6-3-210","volume":"6","author":"F Giorgini","year":"2005","unstructured":"Giorgini F, Muchowski PJ . Connecting the dots in Huntington\u2019s disease with protein interaction networks. Genome Biol 2005; 6: 210.","journal-title":"Genome Biol"},{"key":"BFcddis2012121_CR24","doi-asserted-by":"crossref","first-page":"1261","DOI":"10.1523\/JNEUROSCI.18-04-01261.1998","volume":"18","author":"SH Li","year":"1998","unstructured":"Li SH, Gutekunst CA, Hersch SM, Li XJ . Interaction of huntingtin-associated protein with dynactin P150Glued. J Neurosci 1998; 18: 1261\u20131269.","journal-title":"J Neurosci"},{"key":"BFcddis2012121_CR25","doi-asserted-by":"crossref","first-page":"39271","DOI":"10.1074\/jbc.C100401200","volume":"276","author":"M Metzler","year":"2001","unstructured":"Metzler M, Legendre-Guillemin V, Gan L, Chopra V, Kwok A, McPherson PS et al. HIP1 functions in clathrin-mediated endocytosis through binding to clathrin and adaptor protein 2. J Biol Chem 2001; 276: 39271\u201339276.","journal-title":"J Biol Chem"},{"key":"BFcddis2012121_CR26","doi-asserted-by":"crossref","first-page":"853","DOI":"10.1016\/j.molcel.2004.09.016","volume":"15","author":"H Goehler","year":"2004","unstructured":"Goehler H, Lalowski M, Stelzl U, Waelter S, Stroedicke M, Worm U et al. A protein interaction network links GIT1, an enhancer of huntingtin aggregation, to Huntington\u2019s disease. Mol Cell 2004; 15: 853\u2013865.","journal-title":"Mol Cell"},{"key":"BFcddis2012121_CR27","doi-asserted-by":"crossref","first-page":"24713","DOI":"10.1074\/jbc.M103501200","volume":"276","author":"Y Sun","year":"2001","unstructured":"Sun Y, Savanenin A, Reddy PH, Liu YF . Polyglutamine-expanded huntingtin promotes sensitization of N-methyl-D-aspartate receptors via post-synaptic density 95. J Biol Chem 2001; 276: 24713\u201324718.","journal-title":"J Biol Chem"},{"key":"BFcddis2012121_CR28","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1038\/ng0595-3","volume":"10","author":"D Housman","year":"1995","unstructured":"Housman D . Gain of glutamines, gain of function? Nat Genet 1995; 10: 3\u20134.","journal-title":"Nat Genet"},{"key":"BFcddis2012121_CR29","doi-asserted-by":"crossref","first-page":"524","DOI":"10.1046\/j.1399-0004.2003.00155.x","volume":"64","author":"F Squitieri","year":"2003","unstructured":"Squitieri F, Almqvist EW, Cannella M, Cislaghi G, Hayden MR . Predictive testing for persons at risk for homozygosity for CAG expansion in the Huntington disease gene. Clin Genet 2003; 64: 524\u2013525.","journal-title":"Clin Genet"},{"key":"BFcddis2012121_CR30","doi-asserted-by":"crossref","first-page":"8195","DOI":"10.1128\/MCB.24.18.8195-8209.2004","volume":"24","author":"E Trushina","year":"2004","unstructured":"Trushina E, Dyer RB, Badger JD, Ure D, Eide L, Tran DD et al. Mutant huntingtin impairs axonal trafficking in mammalian neurons in vivo and in vitro. Mol Cell Biol 2004; 24: 8195\u20138209.","journal-title":"Mol Cell Biol"},{"key":"BFcddis2012121_CR31","doi-asserted-by":"crossref","first-page":"294","DOI":"10.1016\/j.pneurobio.2007.01.003","volume":"81","author":"C Zuccato","year":"2007","unstructured":"Zuccato C, Cattaneo E . Role of brain-derived neurotrophic factor in Huntington\u2019s disease. Prog Neurobiol 2007; 81: 294\u2013330.","journal-title":"Prog Neurobiol"},{"key":"BFcddis2012121_CR32","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/S0304-3940(00)00872-7","volume":"282","author":"I Dragatsis","year":"2000","unstructured":"Dragatsis I, Dietrich P, Zeitlin S . Expression of the Huntingtin-associated protein 1 gene in the developing and adult mouse. Neurosci Lett 2000; 282: 37\u201340.","journal-title":"Neurosci Lett"},{"key":"BFcddis2012121_CR33","doi-asserted-by":"crossref","first-page":"e1000838","DOI":"10.1371\/journal.pgen.1000838","volume":"6","author":"S Zheng","year":"2010","unstructured":"Zheng S, Clabough EB, Sarkar S, Futter M, Rubinsztein DC, Zeitlin SO . Deletion of the huntingtin polyglutamine stretch enhances neuronal autophagy and longevity in mice. PLoS Genet 2010; 6: e1000838.","journal-title":"PLoS Genet"},{"key":"BFcddis2012121_CR34","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1093\/hmg\/ddi477","volume":"15","author":"EB Clabough","year":"2006","unstructured":"Clabough EB, Zeitlin SO . Deletion of the triplet repeat encoding polyglutamine within the mouse Huntington\u2019s disease gene results in subtle behavioral\/motor phenotypes in vivo and elevated levels of ATP with cellular senescence in vitro. Hum Mol Genet 2006; 15: 607\u2013623.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR35","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1385\/MN:23:1:21","volume":"23","author":"S Sipione","year":"2001","unstructured":"Sipione S, Cattaneo E . Modeling Huntington\u2019s disease in cells, flies, and mice. Mol Neurobiol 2001; 23: 21\u201351.","journal-title":"Mol Neurobiol"},{"key":"BFcddis2012121_CR36","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1126\/science.1059581","volume":"293","author":"C Zuccato","year":"2001","unstructured":"Zuccato C, Ciammola A, Rigamonti D, Leavitt BR, Goffredo D, Conti L et al. Loss of huntingtin-mediated BDNF gene transcription in Huntington\u2019s disease. Science 2001; 293: 493\u2013498.","journal-title":"Science"},{"key":"BFcddis2012121_CR37","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1186\/1471-2350-10-11","volume":"10","author":"M Cannella","year":"2009","unstructured":"Cannella M, Maglione V, Martino T, Ragona G, Frati L, Li GM et al. DNA instability in replicating Huntington\u2019s disease lymphoblasts. BMC Med Genet 2009; 10: 11.","journal-title":"BMC Med Genet"},{"key":"BFcddis2012121_CR38","doi-asserted-by":"crossref","first-page":"12784","DOI":"10.1073\/pnas.221451398","volume":"98","author":"YJ Kim","year":"2001","unstructured":"Kim YJ, Yi Y, Sapp E, Wang Y, Cuiffo B, Kegel KB et al. Caspase 3-cleaved N-terminal fragments of wild-type and mutant huntingtin are present in normal and Huntington\u2019s disease brains, associate with membranes, and undergo calpain-dependent proteolysis. Proc Natl Acad Sci USA 2001; 98: 12784\u201312789.","journal-title":"Proc Natl Acad Sci USA"},{"key":"BFcddis2012121_CR39","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1016\/j.neuron.2010.07.011","volume":"67","author":"A Johri","year":"2010","unstructured":"Johri A, Beal MF . Hunting-ton for new proteases: MMPs as the new target? Neuron 2010; 67: 171\u2013173.","journal-title":"Neuron"},{"key":"BFcddis2012121_CR40","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1038\/ng0298-150","volume":"18","author":"D Martindale","year":"1998","unstructured":"Martindale D, Hackam A, Wieczorek A, Ellerby L, Wellington C, McCutcheon K et al. Length of huntingtin and its polyglutamine tract influences localization and frequency of intracellular aggregates. Nat Genet 1998; 18: 150\u2013154.","journal-title":"Nat Genet"},{"key":"BFcddis2012121_CR41","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1111\/j.1471-4159.2007.05032.x","volume":"104","author":"A Weiss","year":"2008","unstructured":"Weiss A, Klein C, Woodman B, Sathasivam K, Bibel M, Regulier E et al. Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington\u2019s disease. J Neurochem 2008; 104: 846\u2013858.","journal-title":"J Neurochem"},{"key":"BFcddis2012121_CR42","doi-asserted-by":"crossref","first-page":"1047","DOI":"10.1098\/rstb.1999.0457","volume":"354","author":"AS Hackam","year":"1999","unstructured":"Hackam AS, Hodgson JG, Singaraja R, Zhang T, Gan L, Gutekunst CA et al. Evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington\u2019s disease in cell culture and in transgenic mice expressing mutant huntingtin. Philos Trans R Soc Lond Ser B 1999; 354: 1047\u20131055.","journal-title":"Philos Trans R Soc Lond Ser B"},{"key":"BFcddis2012121_CR43","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1093\/hmg\/8.1.25","volume":"8","author":"AS Hackam","year":"1999","unstructured":"Hackam AS, Singaraja R, Zhang T, Gan L, Hayden MR . In vitro evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington\u2019s disease. Hum Mol Genet 1999; 8: 25\u201333.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR44","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1002\/jgm.1150","volume":"10","author":"B Huang","year":"2008","unstructured":"Huang B, Schiefer J, Sass C, Kosinski CM, Kochanek S . Inducing huntingtin inclusion formation in primary neuronal cell culture and in vivo by high-capacity adenoviral vectors expressing truncated and full-length huntingtin with polyglutamine expansion. J Gene Med 2008; 10: 269\u2013279.","journal-title":"J Gene Med"},{"key":"BFcddis2012121_CR45","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1016\/j.nbd.2005.05.017","volume":"20","author":"D Zala","year":"2005","unstructured":"Zala D, Benchoua A, Brouillet E, Perrin V, Gaillard MC, Zurn AD et al. Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment. Neurobiol Dis 2005; 20: 785\u2013798.","journal-title":"Neurobiol Dis"},{"key":"BFcddis2012121_CR46","doi-asserted-by":"crossref","first-page":"1829","DOI":"10.1093\/hmg\/10.17.1829","volume":"10","author":"A Wyttenbach","year":"2001","unstructured":"Wyttenbach A, Swartz J, Kita H, Thykjaer T, Carmichael J, Bradley J et al. Polyglutamine expansions cause decreased CRE-mediated transcription and early gene expression changes prior to cell death in an inducible cell model of Huntington\u2019s disease. Hum Mol Genet 2001; 10: 1829\u20131845.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR47","doi-asserted-by":"crossref","first-page":"1642","DOI":"10.1016\/S0140-6736(03)13304-1","volume":"361","author":"G Bates","year":"2003","unstructured":"Bates G . Huntingtin aggregation and toxicity in Huntington\u2019s disease. Lancet 2003; 361: 1642\u20131644.","journal-title":"Lancet"},{"key":"BFcddis2012121_CR48","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1007\/s00401-010-0703-0","volume":"120","author":"SL Hands","year":"2010","unstructured":"Hands SL, Wyttenbach A . Neurotoxic protein oligomerisation associated with polyglutamine diseases. Acta Neuropathol 2010; 120: 419\u2013437.","journal-title":"Acta Neuropathol"},{"key":"BFcddis2012121_CR49","doi-asserted-by":"crossref","first-page":"962","DOI":"10.1111\/j.1471-4159.2004.02376.x","volume":"89","author":"MD Kaytor","year":"2004","unstructured":"Kaytor MD, Wilkinson KD, Warren ST . Modulating huntingtin half-life alters polyglutamine-dependent aggregate formation and cell toxicity. J Neurochem 2004; 89: 962\u2013973.","journal-title":"J Neurochem"},{"key":"BFcddis2012121_CR50","doi-asserted-by":"crossref","first-page":"765","DOI":"10.1093\/hmg\/ddi071","volume":"14","author":"MA Poirier","year":"2005","unstructured":"Poirier MA, Jiang H, Ross CA . A structure-based analysis of huntingtin mutant polyglutamine aggregation and toxicity: evidence for a compact beta-sheet structure. Hum Mol Genet 2005; 14: 765\u2013774.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR51","doi-asserted-by":"crossref","first-page":"41032","DOI":"10.1074\/jbc.M205809200","volume":"277","author":"MA Poirier","year":"2002","unstructured":"Poirier MA, Li H, Macosko J, Cai S, Amzel M, Ross CA . Huntingtin spheroids and protofibrils as precursors in polyglutamine fibrilization. J Biol Chem 2002; 277: 41032\u201341037.","journal-title":"J Biol Chem"},{"key":"BFcddis2012121_CR52","doi-asserted-by":"crossref","first-page":"3078","DOI":"10.1016\/j.bpj.2010.02.058","volume":"98","author":"G Ossato","year":"2010","unstructured":"Ossato G, Digman MA, Aiken C, Lukacsovich T, Marsh JL, Gratton E . A two-step path to inclusion formation of huntingtin peptides revealed by number and brightness analysis. Biophys J 2010; 98: 3078\u20133085.","journal-title":"Biophys J"},{"key":"BFcddis2012121_CR53","doi-asserted-by":"crossref","first-page":"13060","DOI":"10.1073\/pnas.181479798","volume":"98","author":"RS Rajan","year":"2001","unstructured":"Rajan RS, Illing ME, Bence NF, Kopito RR . Specificity in intracellular protein aggregation and inclusion body formation. Proc Natl Acad Sci USA 2001; 98: 13060\u201313065.","journal-title":"Proc Natl Acad Sci USA"},{"key":"BFcddis2012121_CR54","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1038\/nature02998","volume":"431","author":"M Arrasate","year":"2004","unstructured":"Arrasate M, Mitra S, Schweitzer ES, Segal MR, Finkbeiner S . Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death. Nature 2004; 431: 805\u2013810.","journal-title":"Nature"},{"key":"BFcddis2012121_CR55","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/S0896-6273(02)00761-4","volume":"35","author":"PJ Muchowski","year":"2002","unstructured":"Muchowski PJ . Protein misfolding, amyloid formation, and neurodegeneration: a critical role for molecular chaperones? Neuron 2002; 35: 9\u201312.","journal-title":"Neuron"},{"key":"BFcddis2012121_CR56","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1093\/hmg\/ddg074","volume":"12","author":"JP Taylor","year":"2003","unstructured":"Taylor JP, Tanaka F, Robitschek J, Sandoval CM, Taye A, Markovic-Plese S et al. Aggresomes protect cells by enhancing the degradation of toxic polyglutamine-containing protein. Hum Mol Genet 2003; 12: 749\u2013757.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR57","doi-asserted-by":"crossref","first-page":"2541","DOI":"10.1016\/j.biocel.2004.02.003","volume":"36","author":"JL Webb","year":"2004","unstructured":"Webb JL, Ravikumar B, Rubinsztein DC . Microtubule disruption inhibits autophagosome\u2013lysosome fusion: implications for studying the roles of aggresomes in polyglutamine diseases. Int J Biochem Cell Biol 2004; 36: 2541\u20132550.","journal-title":"Int J Biochem Cell Biol"},{"key":"BFcddis2012121_CR58","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1002\/ana.410420411","volume":"42","author":"E Sapp","year":"1997","unstructured":"Sapp E, Schwarz C, Chase K, Bhide PG, Young AB, Penney J et al. Huntingtin localization in brains of normal and Huntington\u2019s disease patients. Ann Neurol 1997; 42: 604\u2013612.","journal-title":"Ann Neurol"},{"key":"BFcddis2012121_CR59","doi-asserted-by":"crossref","first-page":"2435","DOI":"10.1097\/00001756-199908200-00001","volume":"10","author":"GH Wang","year":"1999","unstructured":"Wang GH, Mitsui K, Kotliarova S, Yamashita A, Nagao Y, Tokuhiro S et al. Caspase activation during apoptotic cell death induced by expanded polyglutamine in N2a cells. NeuroReport 1999; 10: 2435\u20132438.","journal-title":"NeuroReport"},{"key":"BFcddis2012121_CR60","doi-asserted-by":"crossref","first-page":"R40","DOI":"10.1093\/hmg\/ddn412","volume":"18","author":"M Pennuto","year":"2009","unstructured":"Pennuto M, Palazzolo I, Poletti A . Post-translational modifications of expanded polyglutamine proteins: impact on neurotoxicity. Hum Mol Genet 2009; 18: R40\u201347.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR61","doi-asserted-by":"crossref","first-page":"828","DOI":"10.1016\/j.neuron.2009.11.020","volume":"64","author":"X Gu","year":"2009","unstructured":"Gu X, Greiner ER, Mishra R, Kodali R, Osmand A, Finkbeiner S et al. Serines 13 and 16 are critical determinants of full-length human mutant huntingtin induced disease pathogenesis in HD mice. Neuron 2009; 64: 828\u2013840.","journal-title":"Neuron"},{"key":"BFcddis2012121_CR62","doi-asserted-by":"crossref","first-page":"1424","DOI":"10.1093\/hmg\/ddr023","volume":"20","author":"LS Havel","year":"2011","unstructured":"Havel LS, Wang CE, Wade B, Huang B, Li S, Li XJ . Preferential accumulation of N-terminal mutant huntingtin in the nuclei of striatal neurons is regulated by phosphorylation. Hum Mol Genet 2011; 20: 1424\u20131437.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR63","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1016\/S0968-0004(03)00168-3","volume":"28","author":"P Harjes","year":"2003","unstructured":"Harjes P, Wanker EE . The hunt for huntingtin function: interaction partners tell many different stories. Trends Biochem Sci 2003; 28: 425\u2013433.","journal-title":"Trends Biochem Sci"},{"key":"BFcddis2012121_CR64","doi-asserted-by":"crossref","first-page":"2795","DOI":"10.1093\/hmg\/ddr178","volume":"20","author":"A Davranche","year":"2011","unstructured":"Davranche A, Aviolat H, Zeder-Lutz G, Busso D, Altschuh D, Trottier Y et al. Huntingtin affinity for partners is not changed by polyglutamine length: aggregation itself triggers aberrant interactions. Hum Mol Genet 2011; 20: 2795\u20132806.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR65","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1006\/jmbi.2001.4850","volume":"311","author":"S Chen","year":"2001","unstructured":"Chen S, Berthelier V, Yang W, Wetzel R . Polyglutamine aggregation behavior in vitro supports a recruitment mechanism of cytotoxicity. J Mol Biol 2001; 311: 173\u2013182.","journal-title":"J Mol Biol"},{"key":"BFcddis2012121_CR66","doi-asserted-by":"crossref","first-page":"128","DOI":"10.4161\/pri.1.2.4440","volume":"1","author":"Y Wang","year":"2007","unstructured":"Wang Y, Meriin AB, Costello CE, Sherman MY . Characterization of proteins associated with polyglutamine aggregates: a novel approach towards isolation of aggregates from protein conformation disorders. Prion 2007; 1: 128\u2013135.","journal-title":"Prion"},{"key":"BFcddis2012121_CR67","doi-asserted-by":"crossref","first-page":"1163","DOI":"10.1038\/ncb1478","volume":"8","author":"A Kitamura","year":"2006","unstructured":"Kitamura A, Kubota H, Pack CG, Matsumoto G, Hirayama S, Takahashi Y et al. Cytosolic chaperonin prevents polyglutamine toxicity with altering the aggregation state. Nat Cell Biol 2006; 8: 1163\u20131170.","journal-title":"Nat Cell Biol"},{"key":"BFcddis2012121_CR68","doi-asserted-by":"crossref","first-page":"4307","DOI":"10.1038\/sj.emboj.7600426","volume":"23","author":"CI Holmberg","year":"2004","unstructured":"Holmberg CI, Staniszewski KE, Mensah KN, Matouschek A, Morimoto RI . Inefficient degradation of truncated polyglutamine proteins by the proteasome. EMBO J 2004; 23: 4307\u20134318.","journal-title":"EMBO J"},{"key":"BFcddis2012121_CR69","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1016\/j.molcel.2004.06.029","volume":"15","author":"G Schaffar","year":"2004","unstructured":"Schaffar G, Breuer P, Boteva R, Behrends C, Tzvetkov N, Strippel N et al. Cellular toxicity of polyglutamine expansion proteins: mechanism of transcription factor deactivation. Mol Cell 2004; 15: 95\u2013105.","journal-title":"Mol Cell"},{"key":"BFcddis2012121_CR70","doi-asserted-by":"crossref","first-page":"826","DOI":"10.1038\/ncb863","volume":"4","author":"S Kim","year":"2002","unstructured":"Kim S, Nollen EA, Kitagawa K, Bindokas VP, Morimoto RI . Polyglutamine protein aggregates are dynamic. Nat Cell Biol 2002; 4: 826\u2013831.","journal-title":"Nat Cell Biol"},{"key":"BFcddis2012121_CR71","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1038\/nm0798-755","volume":"4","author":"E Mezey","year":"1998","unstructured":"Mezey E, Dehejia A, Harta G, Papp MI, Polymeropoulos MH, Brownstein MJ . Alpha synuclein in neurodegenerative disorders: murderer or accomplice? Nat Med 1998; 4: 755\u2013757.","journal-title":"Nat Med"},{"key":"BFcddis2012121_CR72","doi-asserted-by":"crossref","first-page":"11282","DOI":"10.1073\/pnas.90.23.11282","volume":"90","author":"K Ueda","year":"1993","unstructured":"Ueda K, Fukushima H, Masliah E, Xia Y, Iwai A, Yoshimoto M et al. Molecular cloning of cDNA encoding an unrecognized component of amyloid in Alzheimer disease. Proc Natl Acad Sci USA 1993; 90: 11282\u201311286.","journal-title":"Proc Natl Acad Sci USA"},{"key":"BFcddis2012121_CR73","doi-asserted-by":"crossref","first-page":"9141","DOI":"10.1073\/pnas.92.20.9141","volume":"92","author":"M Yoshimoto","year":"1995","unstructured":"Yoshimoto M, Iwai A, Kang D, Otero DA, Xia Y, Saitoh T . NACP the precursor protein of the non-amyloid beta\/A4 protein (A beta) component of Alzheimer disease amyloid, binds A beta and stimulates A beta aggregation. Proc Natl Acad Sci USA 1995; 92: 9141\u20139145.","journal-title":"Proc Natl Acad Sci USA"},{"key":"BFcddis2012121_CR74","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/S0304-3940(00)01247-7","volume":"289","author":"V Charles","year":"2000","unstructured":"Charles V, Mezey E, Reddy PH, Dehejia A, Young TA, Polymeropoulos MH et al. Alpha-synuclein immunoreactivity of huntingtin polyglutamine aggregates in striatum and cortex of Huntington\u2019s disease patients and transgenic mouse models. Neurosci Lett 2000; 289: 29\u201332.","journal-title":"Neurosci Lett"},{"key":"BFcddis2012121_CR75","doi-asserted-by":"crossref","first-page":"485","DOI":"10.1093\/hmg\/ddr477","volume":"21","author":"S Corrochano","year":"2012","unstructured":"Corrochano S, Renna M, Carter S, Chrobot N, Kent R, Stewart M et al. Alpha-Synuclein levels modulate Huntington\u2019s disease in mice. Hum Mol Genet 2012; 21: 485\u2013494.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR76","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1093\/hmg\/ddr507","volume":"21","author":"C Tomas-Zapico","year":"2012","unstructured":"Tomas-Zapico C, Diez-Zaera M, Ferrer I, Gomez-Ramos P, Moran MA, Miras-Portugal MT et al. Alpha-Synuclein accumulates in huntingtin inclusions but forms independent filaments and its deficiency attenuates early phenotype in a mouse model of Huntington\u2019s disease. Hum Mol Genet 2012; 21: 495\u2013510.","journal-title":"Hum Mol Genet"},{"issue":"Part 3","key":"BFcddis2012121_CR77","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1042\/bj3460577","volume":"346","author":"RA Furlong","year":"2000","unstructured":"Furlong RA, Narain Y, Rankin J, Wyttenbach A, Rubinsztein DC . Alpha-synuclein overexpression promotes aggregation of mutant huntingtin. Biochem J 2000; 346 (Part 3): 577\u2013581.","journal-title":"Biochem J"},{"key":"BFcddis2012121_CR78","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1016\/j.febslet.2011.11.019","volume":"586","author":"F Herrera","year":"2012","unstructured":"Herrera F, Outeiro TF . Alpha-Synuclein modifies huntingtin aggregation in living cells. FEBS Lett 2012; 586: 7\u201312.","journal-title":"FEBS Lett"},{"key":"BFcddis2012121_CR79","doi-asserted-by":"crossref","first-page":"1089","DOI":"10.1002\/mds.22204","volume":"24","author":"D Caparros-Lefebvre","year":"2009","unstructured":"Caparros-Lefebvre D, Kerdraon O, Devos D, Dhaenens CM, Blum D, Maurage CA et al. Association of corticobasal degeneration and Huntington\u2019s disease: can Tau aggregates protect Huntingtin toxicity? Mov Disord 2009; 24: 1089\u20131090.","journal-title":"Mov Disord"},{"key":"BFcddis2012121_CR80","doi-asserted-by":"crossref","first-page":"149","DOI":"10.1016\/j.neulet.2011.10.070","volume":"506","author":"C Schwab","year":"2012","unstructured":"Schwab C, Yu S, McGeer EG, McGeer PL . Optineurin in Huntington\u2019s disease intranuclear inclusions. Neurosci Lett 2012; 506: 149\u2013154.","journal-title":"Neurosci Lett"},{"key":"BFcddis2012121_CR81","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1007\/s00401-011-0813-3","volume":"121","author":"T Hortobagyi","year":"2011","unstructured":"Hortobagyi T, Troakes C, Nishimura AL, Vance C, van Swieten JC, Seelaar H et al. Optineurin inclusions occur in a minority of TDP-43 positive ALS and FTLD-TDP cases and are rarely observed in other neurodegenerative disorders. Acta Neuropathol 2011; 121: 519\u2013527.","journal-title":"Acta Neuropathol"},{"key":"BFcddis2012121_CR82","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1016\/j.brainresbull.2007.08.005","volume":"75","author":"B Gong","year":"2008","unstructured":"Gong B, Lim MC, Wanderer J, Wyttenbach A, Morton AJ . Time-lapse analysis of aggregate formation in an inducible PC12 cell model of Huntington\u2019s disease reveals time-dependent aggregate formation that transiently delays cell death. Brain Res Bull 2008; 75: 146\u2013157.","journal-title":"Brain Res Bull"},{"key":"BFcddis2012121_CR83","doi-asserted-by":"crossref","first-page":"4830","DOI":"10.1093\/hmg\/ddp455","volume":"18","author":"TL Henshall","year":"2009","unstructured":"Henshall TL, Tucker B, Lumsden AL, Nornes S, Lardelli MT, Richards RI . Selective neuronal requirement for huntingtin in the developing zebrafish. Hum Mol Genet 2009; 18: 4830\u20134842.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR84","doi-asserted-by":"crossref","first-page":"889","DOI":"10.1038\/mp.2011.28","volume":"16","author":"D Soulet","year":"2011","unstructured":"Soulet D, Cicchetti F . The role of immunity in Huntington\u2019s disease. Mol Psychiatry 2011; 16: 889\u2013902.","journal-title":"Mol Psychiatry"},{"key":"BFcddis2012121_CR85","doi-asserted-by":"crossref","first-page":"1715","DOI":"10.1002\/mds.21540","volume":"22","author":"C Turner","year":"2007","unstructured":"Turner C, Cooper JM, Schapira AH . Clinical correlates of mitochondrial function in Huntington\u2019s disease muscle. Mov Disord 2007; 22: 1715\u20131721.","journal-title":"Mov Disord"},{"key":"BFcddis2012121_CR86","doi-asserted-by":"crossref","first-page":"3048","DOI":"10.1093\/hmg\/ddp243","volume":"18","author":"RK Chaturvedi","year":"2009","unstructured":"Chaturvedi RK, Adhihetty P, Shukla S, Hennessy T, Calingasan N, Yang L et al. Impaired PGC-1alpha function in muscle in Huntington\u2019s disease. Hum Mol Genet 2009; 18: 3048\u20133065.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR87","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1046\/j.1471-4159.2003.02009.x","volume":"87","author":"M Orth","year":"2003","unstructured":"Orth M, Cooper JM, Bates GP, Schapira AH . Inclusion formation in Huntington\u2019s disease R6\/2 mouse muscle cultures. J Neurochem 2003; 87: 1\u20136.","journal-title":"J Neurochem"},{"key":"BFcddis2012121_CR88","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1016\/0092-8674(95)90542-1","volume":"81","author":"J Nasir","year":"1995","unstructured":"Nasir J, Floresco SB, O\u2019Kusky JR, Diewert VM, Richman JM, Zeisler J et al. Targeted disruption of the Huntington\u2019s disease gene results in embryonic lethality and behavioral and morphological changes in heterozygotes. Cell 1995; 81: 811\u2013823.","journal-title":"Cell"},{"key":"BFcddis2012121_CR89","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1046\/j.1471-4159.2001.00033.x","volume":"76","author":"JL Mazzola","year":"2001","unstructured":"Mazzola JL, Sirover MA . Reduction of glyceraldehyde-3-phosphate dehydrogenase activity in Alzheimer\u2019s disease and in Huntington\u2019s disease fibroblasts. J Neurochem 2001; 76: 442\u2013449.","journal-title":"J Neurochem"},{"key":"BFcddis2012121_CR90","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1007\/s00702-009-0328-4","volume":"117","author":"F Squitieri","year":"2010","unstructured":"Squitieri F, Falleni A, Cannella M, Orobello S, Fulceri F, Lenzi P et al. Abnormal morphology of peripheral cell tissues from patients with Huntington disease. J Neural Transm 2010; 117: 77\u201383.","journal-title":"J Neural Transm"},{"key":"BFcddis2012121_CR91","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1016\/S0006-8993(99)01451-1","volume":"835","author":"K Manley","year":"1999","unstructured":"Manley K, Pugh J, Messer A . Instability of the CAG repeat in immortalized fibroblast cell cultures from Huntington\u2019s disease transgenic mice. Brain Res 1999; 835: 74\u201379.","journal-title":"Brain Res"},{"key":"BFcddis2012121_CR92","doi-asserted-by":"crossref","first-page":"10199","DOI":"10.1523\/JNEUROSCI.1694-10.2010","volume":"30","author":"ES Maywood","year":"2010","unstructured":"Maywood ES, Fraenkel E, McAllister CJ, Wood N, Reddy AB, Hastings MH et al. Disruption of peripheral circadian timekeeping in a mouse model of Huntington\u2019s disease and its restoration by temporally scheduled feeding. J Neurosci 2010; 30: 10199\u201310204.","journal-title":"J Neurosci"},{"key":"BFcddis2012121_CR93","doi-asserted-by":"crossref","first-page":"1111","DOI":"10.1016\/j.bbadis.2011.05.006","volume":"1812","author":"MC Chiang","year":"2011","unstructured":"Chiang MC, Chern Y, Juo CG . The dysfunction of hepatic transcriptional factors in mice with Huntington\u2019s disease. Biochim Biophys Acta 2011; 1812: 1111\u20131120.","journal-title":"Biochim Biophys Acta"},{"key":"BFcddis2012121_CR94","doi-asserted-by":"crossref","first-page":"3942","DOI":"10.1093\/hmg\/ddp336","volume":"18","author":"R Smith","year":"2009","unstructured":"Smith R, Bacos K, Fedele V, Soulet D, Walz HA, Obermuller S et al. Mutant huntingtin interacts with {beta}-tubulin and disrupts vesicular transport and insulin secretion. Hum Mol Genet 2009; 18: 3942\u20133954.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR95","doi-asserted-by":"crossref","first-page":"1305","DOI":"10.1007\/s00018-011-0692-8","volume":"69","author":"A Cape","year":"2012","unstructured":"Cape A, Chen X, Wang CE, O\u2019Neill A, Lin YF, He J et al. Loss of huntingtin-associated protein 1 impairs insulin secretion from pancreatic beta-cells. Cell Mol Life Sci 2012; 69: 1305\u20131317.","journal-title":"Cell Mol Life Sci"},{"key":"BFcddis2012121_CR96","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1093\/hmg\/9.3.387","volume":"9","author":"M Metzler","year":"2000","unstructured":"Metzler M, Helgason CD, Dragatsis I, Zhang T, Gan L, Pineault N et al. Huntingtin is required for normal hematopoiesis. Hum Mol Genet 2000; 9: 387\u2013394.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR97","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1016\/j.mad.2005.09.010","volume":"127","author":"F Squitieri","year":"2006","unstructured":"Squitieri F, Cannella M, Sgarbi G, Maglione V, Falleni A, Lenzi P et al. Severe ultrastructural mitochondrial changes in lymphoblasts homozygous for Huntington disease mutation. Mech Ageing Dev 2006; 127: 217\u2013220.","journal-title":"Mech Ageing Dev"},{"key":"BFcddis2012121_CR98","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1016\/j.mad.2005.09.011","volume":"127","author":"V Maglione","year":"2006","unstructured":"Maglione V, Cannella M, Gradini R, Cislaghi G, Squitieri F . Huntingtin fragmentation and increased caspase 3, 8 and 9 activities in lymphoblasts with heterozygous and homozygous Huntington\u2019s disease mutation. Mech Ageing Dev 2006; 127: 213\u2013216.","journal-title":"Mech Ageing Dev"},{"key":"BFcddis2012121_CR99","doi-asserted-by":"crossref","first-page":"1599","DOI":"10.1007\/s00702-011-0646-1","volume":"118","author":"F Squitieri","year":"2011","unstructured":"Squitieri F, Maglione V, Orobello S, Fornai F . Genotype-, aging-dependent abnormal caspase activity in Huntington disease blood cells. J Neural Transm 2011; 118: 1599\u20131607.","journal-title":"J Neural Transm"},{"key":"BFcddis2012121_CR100","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1016\/S1474-4422(03)00411-3","volume":"2","author":"D Blum","year":"2003","unstructured":"Blum D, Hourez R, Galas MC, Popoli P, Schiffmann SN . Adenosine receptors and Huntington\u2019s disease: implications for pathogenesis and therapeutics. Lancet Neurol 2003; 2: 366\u2013374.","journal-title":"Lancet Neurol"},{"key":"BFcddis2012121_CR101","first-page":"2148","volume":"17","author":"K Varani","year":"2003","unstructured":"Varani K, Abbracchio MP, Cannella M, Cislaghi G, Giallonardo P, Mariotti C et al. Aberrant A2A receptor function in peripheral blood cells in Huntington\u2019s disease. FASEB J 2003; 17: 2148\u20132150.","journal-title":"FASEB J"},{"key":"BFcddis2012121_CR102","doi-asserted-by":"crossref","first-page":"1001","DOI":"10.1083\/jcb.200508072","volume":"171","author":"JY Shin","year":"2005","unstructured":"Shin JY, Fang ZH, Yu ZX, Wang CE, Li SH, Li XJ . Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity. J Cell Biol 2005; 171: 1001\u20131012.","journal-title":"J Cell Biol"},{"key":"BFcddis2012121_CR103","doi-asserted-by":"crossref","first-page":"22480","DOI":"10.1073\/pnas.0911503106","volume":"106","author":"J Bradford","year":"2009","unstructured":"Bradford J, Shin JY, Roberts M, Wang CE, Li XJ, Li S . Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms. Proc Natl Acad Sci USA 2009; 106: 22480\u201322485.","journal-title":"Proc Natl Acad Sci USA"},{"key":"BFcddis2012121_CR104","doi-asserted-by":"crossref","first-page":"10653","DOI":"10.1074\/jbc.M109.083287","volume":"285","author":"J Bradford","year":"2010","unstructured":"Bradford J, Shin JY, Roberts M, Wang CE, Sheng G, Li S et al. Mutant huntingtin in glial cells exacerbates neurological symptoms of Huntington disease mice. J Biol Chem 2010; 285: 10653\u201310661.","journal-title":"J Biol Chem"},{"key":"BFcddis2012121_CR105","doi-asserted-by":"crossref","first-page":"3277","DOI":"10.1523\/JNEUROSCI.0116-08.2008","volume":"28","author":"SY Chou","year":"2008","unstructured":"Chou SY, Weng JY, Lai HL, Liao F, Sun SH, Tu PH et al. Expanded-polyglutamine huntingtin protein suppresses the secretion and production of a chemokine (CCL5\/RANTES) by astrocytes. J Neurosci 2008; 28: 3277\u20133290.","journal-title":"J Neurosci"},{"key":"BFcddis2012121_CR106","doi-asserted-by":"crossref","first-page":"3053","DOI":"10.1093\/hmg\/ddq212","volume":"19","author":"M Faideau","year":"2010","unstructured":"Faideau M, Kim J, Cormier K, Gilmore R, Welch M, Auregan G et al. In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington\u2019s disease subjects. Hum Mol Genet 2010; 19: 3053\u20133067.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR107","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1038\/aps.2011.162","volume":"33","author":"LL Chen","year":"2012","unstructured":"Chen LL, Wu JC, Wang LH, Wang J, Qin ZH, Difiglia M et al. Rapamycin prevents the mutant huntingtin-suppressed GLT-1 expression in cultured astrocytes. Acta Pharmacol Sin 2012; 33: 385\u2013392.","journal-title":"Acta Pharmacol Sin"},{"key":"BFcddis2012121_CR108","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1016\/j.brainres.2012.01.077","volume":"1449","author":"L Wang","year":"2012","unstructured":"Wang L, Lin F, Wang J, Wu J, Han R, Zhu L et al. Expression of mutant N-terminal huntingtin fragment (htt552-100Q) in astrocytes suppresses the secretion of BDNF. Brain Res 2012; 1449: 69\u201382.","journal-title":"Brain Res"},{"key":"BFcddis2012121_CR109","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1016\/j.neuroimage.2007.02.017","volume":"36","author":"AN Anderson","year":"2007","unstructured":"Anderson AN, Pavese N, Edison P, Tai YF, Hammers A, Gerhard A et al. A systematic comparison of kinetic modelling methods generating parametric maps for [(11)C]-(R)-PK11195. Neuroimage 2007; 36: 28\u201337.","journal-title":"Neuroimage"},{"issue":"Part 7","key":"BFcddis2012121_CR110","doi-asserted-by":"crossref","first-page":"1759","DOI":"10.1093\/brain\/awm044","volume":"130","author":"YF Tai","year":"2007","unstructured":"Tai YF, Pavese N, Gerhard A, Tabrizi SJ, Barker RA, Brooks DJ et al. Microglial activation in presymptomatic Huntington\u2019s disease gene carriers. Brain 2007; 130 (Part 7): 1759\u20131766.","journal-title":"Brain"},{"key":"BFcddis2012121_CR111","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1016\/j.brainresbull.2006.10.029","volume":"72","author":"YF Tai","year":"2007","unstructured":"Tai YF, Pavese N, Gerhard A, Tabrizi SJ, Barker RA, Brooks DJ et al. Imaging microglial activation in Huntington\u2019s disease. Brain Res Bull 2007; 72: 148\u2013151.","journal-title":"Brain Res Bull"},{"key":"BFcddis2012121_CR112","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.1097\/WNR.0b013e32832e34ee","volume":"20","author":"A Silvestroni","year":"2009","unstructured":"Silvestroni A, Faull RL, Strand AD, Moller T . Distinct neuroinflammatory profile in post-mortem human Huntington\u2019s disease. NeuroReport 2009; 20: 1098\u20131103.","journal-title":"NeuroReport"},{"key":"BFcddis2012121_CR113","doi-asserted-by":"crossref","first-page":"1869","DOI":"10.1084\/jem.20080178","volume":"205","author":"M Bjorkqvist","year":"2008","unstructured":"Bjorkqvist M, Wild EJ, Thiele J, Silvestroni A, Andre R, Lahiri N et al. A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington\u2019s disease. J Exp Med 2008; 205: 1869\u20131877.","journal-title":"J Exp Med"},{"key":"BFcddis2012121_CR114","doi-asserted-by":"crossref","first-page":"7390","DOI":"10.1074\/jbc.M708192200","volume":"283","author":"F Giorgini","year":"2008","unstructured":"Giorgini F, Moller T, Kwan W, Zwilling D, Wacker JL, Hong S et al. Histone deacetylase inhibition modulates kynurenine pathway activation in yeast, microglia, and mice expressing a mutant huntingtin fragment. J Biol Chem 2008; 283: 7390\u20137400.","journal-title":"J Biol Chem"},{"key":"BFcddis2012121_CR115","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1016\/S0092-8674(00)80513-9","volume":"90","author":"SW Davies","year":"1997","unstructured":"Davies SW, Turmaine M, Cozens BA, DiFiglia M, Sharp AH, Ross CA et al. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 1997; 90: 537\u2013548.","journal-title":"Cell"},{"key":"BFcddis2012121_CR116","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1016\/j.neurobiolaging.2011.02.015","volume":"33","author":"AD Kraft","year":"2012","unstructured":"Kraft AD, Kaltenbach LS, Lo DC, Harry GJ . Activated microglia proliferate at neurites of mutant huntingtin-expressing neurons. Neurobiol Aging 2012; 33: 621 e617\u2013633.","journal-title":"Neurobiol Aging"},{"key":"BFcddis2012121_CR117","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1002\/glia.10261","volume":"43","author":"L Ma","year":"2003","unstructured":"Ma L, Morton AJ, Nicholson LF . Microglia density decreases with age in a mouse model of Huntington\u2019s disease. Glia 2003; 43: 274\u2013280.","journal-title":"Glia"},{"key":"BFcddis2012121_CR118","doi-asserted-by":"crossref","first-page":"1074","DOI":"10.1002\/glia.20526","volume":"55","author":"DA Simmons","year":"2007","unstructured":"Simmons DA, Casale M, Alcon B, Pham N, Narayan N, Lynch G . Ferritin accumulation in dystrophic microglia is an early event in the development of Huntington\u2019s disease. Glia 2007; 55: 1074\u20131084.","journal-title":"Glia"},{"key":"BFcddis2012121_CR119","doi-asserted-by":"crossref","first-page":"13285","DOI":"10.1523\/JNEUROSCI.4393-08.2008","volume":"28","author":"S Tydlacka","year":"2008","unstructured":"Tydlacka S, Wang CE, Wang X, Li S, Li XJ . Differential activities of the ubiquitin\u2013proteasome system in neurons versus glia may account for the preferential accumulation of misfolded proteins in neurons. J Neurosci 2008; 28: 13285\u201313295.","journal-title":"J Neurosci"},{"key":"BFcddis2012121_CR120","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1038\/nature09788","volume":"472","author":"MA Burguillos","year":"2011","unstructured":"Burguillos MA, Deierborg T, Kavanagh E, Persson A, Hajji N, Garcia-Quintanilla A et al. Caspase signalling controls microglia activation and neurotoxicity. Nature 2011; 472: 319\u2013324.","journal-title":"Nature"},{"key":"BFcddis2012121_CR121","doi-asserted-by":"crossref","first-page":"1179","DOI":"10.1016\/j.cell.2006.04.026","volume":"125","author":"RK Graham","year":"2006","unstructured":"Graham RK, Deng Y, Slow EJ, Haigh B, Bissada N, Lu G et al. Cleavage at the caspase-6 site is required for neuronal dysfunction and degeneration due to mutant huntingtin. Cell 2006; 125: 1179\u20131191.","journal-title":"Cell"},{"key":"BFcddis2012121_CR122","doi-asserted-by":"crossref","first-page":"165","DOI":"10.1016\/j.pneurobio.2006.09.005","volume":"80","author":"M Valenza","year":"2006","unstructured":"Valenza M, Cattaneo E . Cholesterol dysfunction in neurodegenerative diseases: is Huntington\u2019s disease in the list? Prog Neurobiol 2006; 80: 165\u2013176.","journal-title":"Prog Neurobiol"},{"key":"BFcddis2012121_CR123","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1016\/j.cell.2006.09.015","volume":"127","author":"L Cui","year":"2006","unstructured":"Cui L, Jeong H, Borovecki F, Parkhurst CN, Tanese N, Krainc D . Transcriptional repression of PGC-1alpha by mutant huntingtin leads to mitochondrial dysfunction and neurodegeneration. Cell 2006; 127: 59\u201369.","journal-title":"Cell"},{"key":"BFcddis2012121_CR124","doi-asserted-by":"crossref","first-page":"9544","DOI":"10.1523\/JNEUROSCI.1291-11.2011","volume":"31","author":"Z Xiang","year":"2011","unstructured":"Xiang Z, Valenza M, Cui L, Leoni V, Jeong HK, Brilli E et al. Peroxisome-proliferator-activated receptor gamma coactivator 1 alpha contributes to dysmyelination in experimental models of Huntington\u2019s disease. J Neurosci 2011; 31: 9544\u20139553.","journal-title":"J Neurosci"},{"key":"BFcddis2012121_CR125","doi-asserted-by":"crossref","first-page":"2061","DOI":"10.1089\/ars.2006.8.2061","volume":"8","author":"SE Browne","year":"2006","unstructured":"Browne SE, Beal MF . Oxidative damage in Huntington\u2019s disease pathogenesis. Antioxid Redox Signal 2006; 8: 2061\u20132073.","journal-title":"Antioxid Redox Signal"},{"key":"BFcddis2012121_CR126","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1016\/j.cell.2006.09.024","volume":"127","author":"J St-Pierre","year":"2006","unstructured":"St-Pierre J, Drori S, Uldry M, Silvaggi JM, Rhee J, Jager S et al. Suppression of reactive oxygen species and neurodegeneration by the PGC-1 transcriptional coactivators. Cell 2006; 127: 397\u2013408.","journal-title":"Cell"},{"key":"BFcddis2012121_CR127","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.nbd.2011.12.042","volume":"46","author":"S Camnasio","year":"2012","unstructured":"Camnasio S, Carri AD, Lombardo A, Grad I, Mariotti C, Castucci A et al. The first reported generation of several induced pluripotent stem cell lines from homozygous and heterozygous Huntington\u2019s disease patients demonstrates mutation related enhanced lysosomal activity. Neurobiol Dis 2012; 46: 41\u201351.","journal-title":"Neurobiol Dis"},{"key":"BFcddis2012121_CR128","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1016\/j.nbd.2011.12.032","volume":"46","author":"V Castiglioni","year":"2012","unstructured":"Castiglioni V, Onorati M, Rochon C, Cattaneo E . Induced pluripotent stem cell lines from Huntington\u2019s disease mice undergo neuronal differentiation while showing alterations in the lysosomal pathway. Neurobiol Dis 2012; 46: 30\u201340.","journal-title":"Neurobiol Dis"},{"key":"BFcddis2012121_CR129","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1016\/j.mcn.2012.03.011","volume":"50","author":"JJ Ritch","year":"2012","unstructured":"Ritch JJ, Valencia A, Alexander J, Sapp E, Gatune L, Sangrey GR et al. Multiple phenotypes in Huntington disease mouse neural stem cells. Mol Cell Neurosci 2012; 50: 70\u201381.","journal-title":"Mol Cell Neurosci"},{"key":"BFcddis2012121_CR130","doi-asserted-by":"crossref","first-page":"877","DOI":"10.1016\/j.cell.2008.07.041","volume":"134","author":"IH Park","year":"2008","unstructured":"Park IH, Arora N, Huo H, Maherali N, Ahfeldt T, Shimamura A et al. Disease-specific induced pluripotent stem cells. Cell 2008; 134: 877\u2013886.","journal-title":"Cell"},{"key":"BFcddis2012121_CR131","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1136\/jmg.38.7.450","volume":"38","author":"LW Ho","year":"2001","unstructured":"Ho LW, Brown R, Maxwell M, Wyttenbach A, Rubinsztein DC . Wild type Huntingtin reduces the cellular toxicity of mutant Huntingtin in mammalian cell models of Huntington\u2019s disease. J Med Genet 2001; 38: 450\u2013452.","journal-title":"J Med Genet"},{"key":"BFcddis2012121_CR132","doi-asserted-by":"crossref","first-page":"6703","DOI":"10.1074\/jbc.M110338200","volume":"277","author":"C Song","year":"2002","unstructured":"Song C, Perides G, Liu YF . Expression of full-length polyglutamine-expanded Huntingtin disrupts growth factor receptor signaling in rat pheochromocytoma (PC12) cells. J Biol Chem 2002; 277: 6703\u20136707.","journal-title":"J Biol Chem"},{"key":"BFcddis2012121_CR133","doi-asserted-by":"crossref","first-page":"319","DOI":"10.1038\/nchembio792","volume":"2","author":"DW Colby","year":"2006","unstructured":"Colby DW, Cassady JP, Lin GC, Ingram VM, Wittrup KD . Stochastic kinetics of intracellular huntingtin aggregate formation. Nat Chem Biol 2006; 2: 319\u2013323.","journal-title":"Nat Chem Biol"},{"key":"BFcddis2012121_CR134","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1093\/hmg\/ddm311","volume":"17","author":"T Takahashi","year":"2008","unstructured":"Takahashi T, Kikuchi S, Katada S, Nagai Y, Nishizawa M, Onodera O . Soluble polyglutamine oligomers formed prior to inclusion body formation are cytotoxic. Hum Mol Genet 2008; 17: 345\u2013356.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR135","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1016\/j.neulet.2008.05.119","volume":"442","author":"C Ye","year":"2008","unstructured":"Ye C, Zhang Y, Wang W, Wang J, Li H . Inhibition of neurite outgrowth and promotion of cell death by cytoplasmic soluble mutant huntingtin stably transfected in mouse neuroblastoma cells. Neurosci Lett 2008; 442: 63\u201368.","journal-title":"Neurosci Lett"},{"key":"BFcddis2012121_CR136","doi-asserted-by":"crossref","first-page":"21807","DOI":"10.1074\/jbc.M109.084434","volume":"285","author":"MA Olshina","year":"2010","unstructured":"Olshina MA, Angley LM, Ramdzan YM, Tang J, Bailey MF, Hill AF et al. Tracking mutant huntingtin aggregation kinetics in cells reveals three major populations that include an invariant oligomer pool. J Biol Chem 2010; 285: 21807\u201321816.","journal-title":"J Biol Chem"},{"key":"BFcddis2012121_CR137","doi-asserted-by":"crossref","first-page":"1294","DOI":"10.1016\/j.bbrc.2009.10.139","volume":"390","author":"HQ Wang","year":"2009","unstructured":"Wang HQ, Xu YX, Zhao XY, Zhao H, Yan J, Sun XB et al. Overexpression of F(0)F(1)-ATP synthase alpha suppresses mutant huntingtin aggregation and toxicity in vitro. Biochem Biophys Res Commun 2009; 390: 1294\u20131298.","journal-title":"Biochem Biophys Res Commun"},{"key":"BFcddis2012121_CR138","doi-asserted-by":"crossref","first-page":"737","DOI":"10.1007\/s10571-011-9679-0","volume":"31","author":"G Dong","year":"2011","unstructured":"Dong G, Ferguson JM, Duling AJ, Nicholas RG, Zhang D, Rezvani K et al. Modeling pathogenesis of Huntington's disease with inducible neuroprogenitor cells. Cell Mol Neurobiol 2011; 31: 737\u2013747.","journal-title":"Cell Mol Neurobiol"},{"key":"BFcddis2012121_CR139","doi-asserted-by":"crossref","first-page":"10541","DOI":"10.1523\/JNEUROSCI.0146-10.2010","volume":"30","author":"J Miller","year":"2010","unstructured":"Miller J, Arrasate M, Shaby BA, Mitra S, Masliah E, Finkbeiner S . Quantitative relationships between huntingtin levels, polyglutamine length, inclusion body formation, and neuronal death provide novel insight into huntington's disease molecular pathogenesis. J Neurosci 2010; 30: 10541\u201310550.","journal-title":"J Neurosci"},{"key":"BFcddis2012121_CR140","doi-asserted-by":"crossref","first-page":"1355","DOI":"10.1093\/hmg\/7.9.1355","volume":"7","author":"A Lunkes","year":"1998","unstructured":"Lunkes A, Mandel JL . A cellular model that recapitulates major pathogenic steps of Huntington\u2019s disease. Hum Mol Genet 1998; 7: 1355\u20131361.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR141","doi-asserted-by":"crossref","first-page":"1953","DOI":"10.1093\/hmg\/11.17.1953","volume":"11","author":"S Sipione","year":"2002","unstructured":"Sipione S, Rigamonti D, Valenza M, Zuccato C, Conti L, Pritchard J et al. Early transcriptional profiles in huntingtin-inducible striatal cells by microarray analyses. Hum Mol Genet 2002; 11: 1953\u20131965.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR142","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1097\/00001756-200303240-00007","volume":"14","author":"S Igarashi","year":"2003","unstructured":"Igarashi S, Morita H, Bennett KM, Tanaka Y, Engelender S, Peters MF et al. Inducible PC12 cell model of Huntington\u2019s disease shows toxicity and decreased histone acetylation. Neuroreport 2003; 14: 565\u2013568.","journal-title":"Neuroreport"},{"key":"BFcddis2012121_CR143","doi-asserted-by":"crossref","first-page":"546","DOI":"10.1016\/j.nbd.2004.04.001","volume":"16","author":"CT Aiken","year":"2004","unstructured":"Aiken CT, Tobin AJ, Schweitzer ES . A cell-based screen for drugs to treat Huntington's disease. Neurobiol Dis 2004; 16: 546\u2013555.","journal-title":"Neurobiol Dis"},{"key":"BFcddis2012121_CR144","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1186\/1750-1326-4-11","volume":"4","author":"A Weiss","year":"2009","unstructured":"Weiss A, Roscic A, Paganetti P . Inducible mutant huntingtin expression in HN10 cells reproduces Huntington\u2019s disease-like neuronal dysfunction. Mol Neurodegener 2009; 4: 11.","journal-title":"Mol Neurodegener"},{"key":"BFcddis2012121_CR145","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1159\/000212174","volume":"23","author":"S Podolsky","year":"1977","unstructured":"Podolsky S, Leopold NA . Abnormal glucose tolerance and arginine tolerance tests in Huntington\u2019s disease. Gerontology 1977; 23: 55\u201363.","journal-title":"Gerontology"},{"key":"BFcddis2012121_CR146","doi-asserted-by":"crossref","first-page":"e8025","DOI":"10.1371\/journal.pone.0008025","volume":"4","author":"H Moffitt","year":"2009","unstructured":"Moffitt H, McPhail GD, Woodman B, Hobbs C, Bates GP . Formation of polyglutamine inclusions in a wide range of non-CNS tissues in the HdhQ150 knock-in mouse model of Huntington's disease. PLoS One 2009; 4: e8025.","journal-title":"PLoS One"},{"key":"BFcddis2012121_CR147","doi-asserted-by":"crossref","first-page":"532","DOI":"10.1007\/BF01307576","volume":"37","author":"HS Garewal","year":"1992","unstructured":"Garewal HS, Leibovitz A, Sampliner RE, Ramsey L, Hendrix MJ, Sloan D . Tissue culture of epithelial cells from esophageal specialized columnar epithelium (Barrett\u2019s esophagus). Dig Dis Sci 1992; 37: 532\u2013536.","journal-title":"Dig Dis Sci"},{"key":"BFcddis2012121_CR148","doi-asserted-by":"crossref","first-page":"3092","DOI":"10.1111\/j.1460-9568.2004.03783.x","volume":"20","author":"RR Ribchester","year":"2004","unstructured":"Ribchester RR, Thomson D, Wood NI, Hinks T, Gillingwater TH, Wishart TM et al. Progressive abnormalities in skeletal muscle and neuromuscular junctions of transgenic mice expressing the Huntington's disease mutation. Eur J Neurosci 2004; 20: 3092\u20133114.","journal-title":"Eur J Neurosci"},{"key":"BFcddis2012121_CR149","doi-asserted-by":"crossref","first-page":"2068","DOI":"10.1038\/sj.cdd.4401967","volume":"13","author":"A Ciammola","year":"2006","unstructured":"Ciammola A, Sassone J, Alberti L, Meola G, Mancinelli E, Russo MA et al. Increased apoptosis, Huntingtin inclusions and altered differentiation in muscle cell cultures from Huntington\u2019s disease subjects. Cell Death Differ 2006; 13: 2068\u20132078.","journal-title":"Cell Death Differ"},{"key":"BFcddis2012121_CR150","doi-asserted-by":"crossref","first-page":"770","DOI":"10.1111\/j.1365-2826.2009.01898.x","volume":"21","author":"TW Boesgaard","year":"2009","unstructured":"Boesgaard TW, Nielsen TT, Josefsen K, Hansen T, Jorgensen T, Pedersen O et al. Huntington\u2019s disease does not appear to increase the risk of diabetes mellitus. J Neuroendocrinol 2009; 21: 770\u2013776.","journal-title":"J Neuroendocrinol"},{"key":"BFcddis2012121_CR151","doi-asserted-by":"crossref","first-page":"512","DOI":"10.1016\/j.nbd.2007.01.006","volume":"26","author":"JM Van Raamsdonk","year":"2007","unstructured":"Van Raamsdonk JM, Murphy Z, Selva DM, Hamidizadeh R, Pearson J, Petersen A et al. Testicular degeneration in Huntington disease. Neurobiol Dis 2007a; 26: 512\u2013520.","journal-title":"Neurobiol Dis"},{"key":"BFcddis2012121_CR152","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1016\/j.nbd.2006.12.010","volume":"26","author":"JM Van Raamsdonk","year":"2007","unstructured":"Van Raamsdonk JM, Metzler M, Slow E, Pearson J, Schwab C, Carroll J et al. Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain. Neurobiol Dis 2007b; 26: 189\u2013200.","journal-title":"Neurobiol Dis"},{"key":"BFcddis2012121_CR153","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1016\/j.bbrc.2008.07.009","volume":"374","author":"S Almeida","year":"2008","unstructured":"Almeida S, Sarmento-Ribeiro AB, Januario C, Rego AC, Oliveira CR . Evidence of apoptosis and mitochondrial abnormalities in peripheral blood cells of Huntington\u2019s disease patients. Biochem Biophys Res Commun 2008; 374: 599\u2013603.","journal-title":"Biochem Biophys Res Commun"},{"key":"BFcddis2012121_CR154","doi-asserted-by":"crossref","first-page":"2743","DOI":"10.1161\/CIRCULATIONAHA.107.750232","volume":"117","author":"JS Pattison","year":"2008","unstructured":"Pattison JS, Sanbe A, Maloyan A, Osinska H, Klevitsky R, Robbins J . Cardiomyocyte expression of a polyglutamine preamyloid oligomer causes heart failure. Circulation 2008; 117: 2743\u20132751.","journal-title":"Circulation"},{"key":"BFcddis2012121_CR155","doi-asserted-by":"crossref","first-page":"813","DOI":"10.1093\/hmg\/8.5.813","volume":"8","author":"K Sathasivam","year":"1999","unstructured":"Sathasivam K, Hobbs C, Turmaine M, Mangiarini L, Mahal A, Bertaux F et al. Formation of polyglutamine inclusions in non-CNS tissue. Hum Mol Genet 1999; 8: 813\u2013822.","journal-title":"Hum Mol Genet"},{"key":"BFcddis2012121_CR156","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1016\/j.nbd.2006.09.016","volume":"25","author":"MJ Mihm","year":"2007","unstructured":"Mihm MJ, Amann DM, Schanbacher BL, Altschuld RA, Bauer JA, Hoyt KR . Cardiac dysfunction in the R6\/2 mouse model of Huntington\u2019s disease. Neurobiol Dis 2007; 25: 297\u2013308.","journal-title":"Neurobiol Dis"},{"key":"BFcddis2012121_CR157","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1161\/CIRCULATIONAHA.109.881433","volume":"122","author":"Y Yoshida","year":"2010","unstructured":"Yoshida Y, Yamanaka S . Recent stem cell advances: induced pluripotent stem cells for disease modeling and stem cell-based regeneration. Circulation 2010; 122: 80\u201387.","journal-title":"Circulation"},{"key":"BFcddis2012121_CR158","doi-asserted-by":"crossref","first-page":"45","DOI":"10.1007\/s11626-007-9079-4","volume":"44","author":"P Sreejit","year":"2008","unstructured":"Sreejit P, Kumar S, Verma RS . An improved protocol for primary culture of cardiomyocyte from neonatal mice. In Vitro Cell Dev Biol Anim 2008; 44: 45\u201350.","journal-title":"In Vitro Cell Dev Biol Anim"}],"container-title":["Cell Death & Disease"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.nature.com\/articles\/cddis2012121.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.nature.com\/articles\/cddis2012121","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/cddis2012121.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,4,28]],"date-time":"2024-04-28T20:18:40Z","timestamp":1714335520000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/cddis2012121"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,8,30]]},"references-count":158,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2012,8]]}},"alternative-id":["BFcddis2012121"],"URL":"https:\/\/doi.org\/10.1038\/cddis.2012.121","relation":{},"ISSN":["2041-4889"],"issn-type":[{"value":"2041-4889","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,8,30]]},"assertion":[{"value":"1 June 2012","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"25 June 2012","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"25 June 2012","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"30 August 2012","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The authors declare no conflict of interest.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}]}}