{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T18:10:56Z","timestamp":1791310256667,"version":"4.3.1"},"reference-count":84,"publisher":"Elsevier BV","issue":"2-3","license":[{"start":{"date-parts":[[2012,6,1]],"date-time":"2012-06-01T00:00:00Z","timestamp":1338508800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2012,6,1]],"date-time":"2012-06-01T00:00:00Z","timestamp":1338508800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Brain Research Bulletin"],"published-print":{"date-parts":[[2012,6]]},"DOI":"10.1016\/j.brainresbull.2011.07.009","type":"journal-article","created":{"date-parts":[[2011,7,25]],"date-time":"2011-07-25T14:46:47Z","timestamp":1311605207000},"page":"104-112","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":36,"title":["Light and electron microscopic characterization of the evolution of cellular pathology in the R6\/1 Huntington's disease transgenic mice"],"prefix":"10.1016","volume":"88","author":[{"given":"Zubeyde","family":"Bayram-Weston","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lesley","family":"Jones","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen B.","family":"Dunnett","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon P.","family":"Brooks","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.brainresbull.2011.07.009_bib0005","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1002\/ar.1090940210","article-title":"Estimation of nuclear population from microtome sections","author":"Abercrombie","year":"1946","journal-title":"Anat. Rec."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0010","doi-asserted-by":"crossref","first-page":"805","DOI":"10.1038\/nature02998","article-title":"Inclusion body formation reduces levels of mutant huntingtin and the risk of neuronal death","volume":"431","author":"Arrasate","year":"2004","journal-title":"Nature"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0015","series-title":"Huntington's Disease","author":"Bates","year":"2002"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0020","doi-asserted-by":"crossref","first-page":"1633","DOI":"10.1093\/hmg\/6.10.1633","article-title":"Transgenic models of Huntington's disease","volume":"6","author":"Bates","year":"1997","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0425","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1016\/j.brainresbull.2011.03.013","article-title":"Light and electron microscopic characterization of the evolution of cellular pathology in HdhQ92\/Q92 Huntington's disease mice","volume":"88","author":"Bayram-Weston","year":"2012","journal-title":"Brain Res. Bull."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0430","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1016\/j.brainresbull.2011.05.005","article-title":"Light and electron microscopic characterization of the evolution of cellular pathology in YAC128 transgenic mice","volume":"88","author":"Bayram-Weston","year":"2012","journal-title":"Brain Res. Bull."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0435","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1016\/j.brainresbull.2011.03.014","article-title":"Light and electron microscopic characterization of the evolution of cellular pathology in the Hdh(CAG)150 Huntington's disease knock-in mouse","volume":"88","author":"Bayram-Weston","year":"2012","journal-title":"Brain Res. Bull."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0040","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/j.brainres.2004.01.021","article-title":"Early exploratory behavior abnormalities in R6\/1 Huntington's disease transgenic mice","volume":"1005","author":"Bolivar","year":"2004","journal-title":"Brain Res."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0045","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1016\/j.bbr.2006.09.007","article-title":"Assessing autism-like behavior in mice: variations in social interactions among inbred strains","volume":"176","author":"Bolivar","year":"2007","journal-title":"Behav. Brain Res."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0050","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1006\/cbir.1993.1075","article-title":"Apoptosis or programmed cell death?","volume":"17","author":"Bowen","year":"1993","journal-title":"Cell Biol. Int."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0055","doi-asserted-by":"crossref","first-page":"796","DOI":"10.1038\/nature05293","article-title":"Cell death in the nervous system","volume":"443","author":"Bredesen","year":"2006","journal-title":"Nature"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0060","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1016\/j.brainresbull.2011.01.010","article-title":"Longitudinal analysis of the behavioural phenotype in R6\/1 (C57BL\/6J) Huntington's disease transgenic mice","volume":"88","author":"Brooks","year":"2012","journal-title":"Brain Res. Bull."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0065","doi-asserted-by":"crossref","first-page":"3248","DOI":"10.1523\/JNEUROSCI.19-08-03248.1999","article-title":"Characterization of progressive motor deficits in mice transgenic for the human Huntington's disease mutation","volume":"19","author":"Carter","year":"1999","journal-title":"J. Neurosci."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0070","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.bbadis.2009.07.012","article-title":"Mitochondria in Huntington's disease","volume":"1802","author":"Damiano","year":"2010","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0075","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1016\/S0092-8674(00)80513-9","article-title":"Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation","volume":"90","author":"Davies","year":"1997","journal-title":"Cell"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0080","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1016\/j.brainresbull.2011.01.012","article-title":"Proteomic changes in the brains of Huntington's disease mouse models reflect pathology and implicate mitochondrial changes","volume":"88","author":"Deschepper","year":"2012","journal-title":"Brain Res. Bull."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0085","doi-asserted-by":"crossref","first-page":"1990","DOI":"10.1126\/science.277.5334.1990","article-title":"Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain","volume":"277","author":"DiFiglia","year":"1997","journal-title":"Science"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0090","first-page":"491","article-title":"Immunohistochemical findings in Huntington's Chorea: report of 9 cases","volume":"88","author":"Galatioto","year":"1996","journal-title":"Pathologica"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0095","doi-asserted-by":"crossref","first-page":"320","DOI":"10.1002\/1531-8257(199903)14:2<320::AID-MDS1018>3.0.CO;2-Z","article-title":"Differential clinical and motor control function in a pair of monozygotic twins with Huntington's disease","volume":"14","author":"Georgiou","year":"1999","journal-title":"Mov. Disord."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0100","doi-asserted-by":"crossref","first-page":"2263","DOI":"10.1111\/j.1582-4934.2008.00402.x","article-title":"Neuronal cell death in neurodegenerative diseases: recurring themes around protein handling","volume":"12","author":"Gorman","year":"2008","journal-title":"J. Cell Mol. Med."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0105","doi-asserted-by":"crossref","first-page":"1823","DOI":"10.1097\/00001756-199806010-00028","article-title":"Neuronal distribution of intranuclear inclusions in Huntington's disease with adult onset","volume":"9","author":"Gourfinkel-An","year":"1998","journal-title":"Neuroreport"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0110","doi-asserted-by":"crossref","first-page":"770","DOI":"10.1126\/science.3155875","article-title":"Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease","volume":"227","author":"Graveland","year":"1985","journal-title":"Science"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0115","doi-asserted-by":"crossref","first-page":"2081","DOI":"10.1111\/j.1460-9568.2005.04365.x","article-title":"Cognitive disorders and neurogenesis deficits in Huntington's disease mice are rescued by fluoxetine","volume":"22","author":"Grote","year":"2005","journal-title":"Eur. J. Neurosci."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0120","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1186\/gm80","article-title":"Huntington's disease: the case for genetic modifiers","volume":"1","author":"Gusella","year":"2009","journal-title":"Genome Med."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0125","doi-asserted-by":"crossref","first-page":"2522","DOI":"10.1523\/JNEUROSCI.19-07-02522.1999","article-title":"Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology","volume":"19","author":"Gutekunst","year":"1999","journal-title":"J. Neurosci."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0130","doi-asserted-by":"crossref","first-page":"1492","DOI":"10.1046\/j.0953-816x.2001.01767.x","article-title":"Resistance to NMDA toxicity correlates with appearance of nuclear inclusions, behavioural deficits and changes in calcium homeostasis in mice transgenic for exon 1 of the huntington gene","volume":"14","author":"Hansson","year":"2001","journal-title":"Eur. J. Neurosci."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0135","doi-asserted-by":"crossref","first-page":"3702","DOI":"10.1093\/hmg\/ddq285","article-title":"Early autophagic response in a novel knock-in model of Huntington disease","volume":"19","author":"Heng","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0140","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/j.jns.2008.11.005","article-title":"Huntington disease in subjects from an Israeli Karaite community carrying alleles of intermediate and expanded CAG repeats in the HTT gene: Huntington disease or phenocopy?","volume":"277","author":"Herishanu","year":"2009","journal-title":"J. Neurol. Sci."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0145","doi-asserted-by":"crossref","first-page":"1702","DOI":"10.1212\/01.WNL.0000098878.47789.BD","article-title":"Profile of cognitive progression in early Huntington's disease","volume":"61","author":"Ho","year":"2003","journal-title":"Neurology"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0150","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1017\/S0033291799002871","article-title":"The molecular biology of Huntington's disease","volume":"31","author":"Ho","year":"2001","journal-title":"Psychol. Med."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0155","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1111\/j.1601-183X.2007.00350.x","article-title":"Brain gene expression correlates with changes in behavior in the R6\/1 mouse model of Huntington's disease","volume":"7","author":"Hodges","year":"2008","journal-title":"Genes Brain Behav."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0160","doi-asserted-by":"crossref","first-page":"78","DOI":"10.1016\/0014-4886(84)90162-6","article-title":"Reduced Purkinje cell density in Huntington's disease","volume":"85","author":"Jeste","year":"1984","journal-title":"Exp. Neurol."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0165","series-title":"Methods in Cell Biology","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0091-679X(08)61921-4","article-title":"Anatomical methods in cell death","author":"Kerr","year":"1995"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0170","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1038\/bjc.1972.33","article-title":"Apoptosis: a basic biological phenomenon with wide-ranging implications in tissue kinetics","volume":"26","author":"Kerr","year":"1972","journal-title":"Br. J. Cancer"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0175","doi-asserted-by":"crossref","first-page":"3919","DOI":"10.1093\/hmg\/ddq306","article-title":"Mitochondrial loss, dysfunction and altered dynamics in Huntington's disease","volume":"19","author":"Kim","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0180","doi-asserted-by":"crossref","first-page":"1013","DOI":"10.1038\/nrd2755","article-title":"Cell death and endoplasmic reticulum stress: disease relevance and therapeutic opportunities","volume":"7","author":"Kim","year":"2008","journal-title":"Nat. Rev. Drug Discov."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0185","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1136\/jnnp.69.6.773","article-title":"Subtle changes among presymptomatic carriers of the Huntington's disease gene","volume":"69","author":"Kirkwood","year":"2000","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"issue":"Pt 7","key":"10.1016\/j.brainresbull.2011.07.009_bib0190","doi-asserted-by":"crossref","first-page":"1329","DOI":"10.1093\/brain\/121.7.1329","article-title":"Evidence for specific cognitive deficits in preclinical Huntington's disease","volume":"121","author":"Lawrence","year":"1998","journal-title":"Brain"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0195","doi-asserted-by":"crossref","first-page":"1829","DOI":"10.1111\/j.1460-9568.2006.04715.x","article-title":"Neurogenesis in the R6\/1 transgenic mouse model of Huntington's disease: effects of environmental enrichment","volume":"23","author":"Lazic","year":"2006","journal-title":"Eur. J. Neurosci."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0200","doi-asserted-by":"crossref","first-page":"21647","DOI":"10.1074\/jbc.M109.016923","article-title":"Monoclonal antibodies recognize distinct conformational epitopes formed by polyglutamine in a mutant huntingtin fragment","volume":"284","author":"Legleiter","year":"2009","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0205","doi-asserted-by":"crossref","first-page":"935","DOI":"10.1007\/s00415-004-0461-9","article-title":"Cognitive changes in patients with Huntington's disease (HD) and asymptomatic carriers of the HD mutation\u2014a longitudinal follow-up study","volume":"251","author":"Lemiere","year":"2004","journal-title":"J. Neurol."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0210","doi-asserted-by":"crossref","first-page":"2021","DOI":"10.1093\/hmg\/ddg218","article-title":"Abnormal association of mutant huntingtin with synaptic vesicles inhibits glutamate release","volume":"12","author":"Li","year":"2003","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0215","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1093\/hmg\/10.2.137","article-title":"Neurological abnormalities in a knock-in mouse model of Huntington's disease","volume":"10","author":"Lin","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0220","doi-asserted-by":"crossref","first-page":"10428","DOI":"10.1523\/JNEUROSCI.19-23-10428.1999","article-title":"Selective discrimination learning impairments in mice expressing the human Huntington's disease mutation","volume":"19","author":"Lione","year":"1999","journal-title":"J. Neurosci."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0225","doi-asserted-by":"crossref","first-page":"2015","DOI":"10.1093\/hmg\/ddl125","article-title":"Genetic background modifies nuclear mutant huntingtin accumulation and HD CAG repeat instability in Huntington's disease knock-in mice","volume":"15","author":"Lloret","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0230","doi-asserted-by":"crossref","first-page":"129","DOI":"10.1097\/00005072-199902000-00003","article-title":"Distribution of inclusions in neuronal nuclei and dystrophic neurites in Huntington disease brain","volume":"58","author":"Maat-Schieman","year":"1999","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0235","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1111\/j.1750-3639.2006.00040.x","article-title":"Neuronal intranuclear and neuropil inclusions for pathological assessment of Huntington's disease","volume":"17","author":"Maat-Schieman","year":"2007","journal-title":"Brain Pathol."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0240","first-page":"3","article-title":"Apoptosis, oncosis, and necrosis: an overview of cell death","volume":"146","author":"Majno","year":"1995","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0245","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1038\/ng0297-197","article-title":"Instability of highly expanded CAG repeats in mice transgenic for the Huntington's disease mutation","volume":"15","author":"Mangiarini","year":"1997","journal-title":"Nat. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0250","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1016\/S0092-8674(00)81369-0","article-title":"Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice","volume":"87","author":"Mangiarini","year":"1996","journal-title":"Cell"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0255","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1038\/nn.2528","article-title":"Cargo recognition failure is responsible for inefficient autophagy in Huntington's disease","volume":"13","author":"Martinez-Vicente","year":"2010","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0260","doi-asserted-by":"crossref","first-page":"8266","DOI":"10.1523\/JNEUROSCI.22-18-08266.2002","article-title":"Early motor dysfunction and striosomal distribution of huntingtin microaggregates in Huntington's disease knock-in mice","volume":"22","author":"Menalled","year":"2002","journal-title":"J. Neurosci."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0265","doi-asserted-by":"crossref","first-page":"10541","DOI":"10.1523\/JNEUROSCI.0146-10.2010","article-title":"Quantitative relationships between huntingtin levels, polyglutamine length, inclusion body formation, and neuronal death provide novel insight into huntington's disease molecular pathogenesis","volume":"30","author":"Miller","year":"2010","journal-title":"J. Neurosci."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0270","doi-asserted-by":"crossref","first-page":"1690","DOI":"10.1093\/hmg\/ddl092","article-title":"Early development of aberrant synaptic plasticity in a mouse model of Huntington's disease","volume":"15","author":"Milnerwood","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0275","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1016\/j.nbd.2008.11.015","article-title":"Paradoxical delay in the onset of disease caused by super-long CAG repeat expansions in R6\/2 mice","volume":"33","author":"Morton","year":"2009","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0280","doi-asserted-by":"crossref","first-page":"1049","DOI":"10.1016\/j.neuroscience.2003.08.053","article-title":"Molecular and behavioral analysis of the R6\/1 Huntington's disease transgenic mouse","volume":"122","author":"Naver","year":"2003","journal-title":"Neuroscience"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0285","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1016\/j.nbd.2007.11.006","article-title":"Gene\u2013environment interactions modulating cognitive function and molecular correlates of synaptic plasticity in Huntington's disease transgenic mice","volume":"29","author":"Nithianantharajah","year":"2008","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0290","series-title":"The Mouse Brain in Stereotaxic Coordinates","author":"Paxinos","year":"2001"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0295","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1006\/exnr.2002.7885","article-title":"Maintenance of susceptibility to neurodegeneration following intrastriatal injections of quinolinic acid in a new transgenic mouse model of Huntington's disease","volume":"175","author":"Petersen","year":"2002","journal-title":"Exp. Neurol."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0300","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1006\/nbdi.2002.0534","article-title":"Evidence for dysfunction of the nigrostriatal pathway in the R6\/1 line of transgenic Huntington's disease mice","volume":"11","author":"Petersen","year":"2002","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0305","doi-asserted-by":"crossref","first-page":"585","DOI":"10.1038\/ng1362","article-title":"Inhibition of mTOR induces autophagy and reduces toxicity of polyglutamine expansions in fly and mouse models of Huntington disease","volume":"36","author":"Ravikumar","year":"2004","journal-title":"Nat. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0310","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1038\/2510","article-title":"Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA","volume":"20","author":"Reddy","year":"1998","journal-title":"Nat. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0315","doi-asserted-by":"crossref","first-page":"147","DOI":"10.1016\/0022-510X(81)90049-6","article-title":"Cerebellar atrophy in Huntington's disease","volume":"50","author":"Rodda","year":"1981","journal-title":"J. Neurol. Sci."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0320","doi-asserted-by":"crossref","first-page":"1615","DOI":"10.1212\/01.WNL.0000065888.88988.6E","article-title":"Evidence for more widespread cerebral pathology in early HD: an MRI-based morphometric analysis","volume":"60","author":"Rosas","year":"2003","journal-title":"Neurology"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0325","doi-asserted-by":"crossref","first-page":"1147","DOI":"10.1016\/S0896-6273(00)80405-5","article-title":"Intranuclear neuronal inclusions: a common pathogenic mechanism for glutamine-repeat neurodegenerative diseases?","volume":"19","author":"Ross","year":"1997","journal-title":"Neuron"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0330","doi-asserted-by":"crossref","first-page":"1003","DOI":"10.1111\/j.1750-3639.1997.tb00898.x","article-title":"Huntington's disease and dentatorubral-pallidoluysian atrophy: proteins, pathogenesis and pathology","volume":"7","author":"Ross","year":"1997","journal-title":"Brain Pathol."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0335","doi-asserted-by":"crossref","first-page":"780","DOI":"10.1038\/nature05291","article-title":"The roles of intracellular protein-degradation pathways in neurodegeneration","volume":"443","author":"Rubinsztein","year":"2006","journal-title":"Nature"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0340","first-page":"16","article-title":"Phenotypic characterization of individuals with 30\u201340 CAG repeats in the Huntington disease (HD) gene reveals HD cases with 36 repeats and apparently normal elderly individuals with 36\u201339 repeats","volume":"59","author":"Rubinsztein","year":"1996","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0345","first-page":"265","article-title":"Intracellular inclusions pathological markers in diseases caused by expanded polyglutamine tracts?","volume":"36","author":"Rubinsztein","year":"1999","journal-title":"J. Med. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0350","doi-asserted-by":"crossref","first-page":"1129","DOI":"10.1590\/S0100-879X2006000800016","article-title":"Striatal and extrastriatal atrophy in Huntington's disease and its relationship with length of the CAG repeat","volume":"39","author":"Ruocco","year":"2006","journal-title":"Braz. J. Med. Biol. Res."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0355","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1002\/ana.410420411","article-title":"Huntingtin localization in brains of normal and Huntington's disease patients","volume":"42","author":"Sapp","year":"1997","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0360","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/S0092-8674(00)81782-1","article-title":"Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions","volume":"95","author":"Saudou","year":"1998","journal-title":"Cell"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0365","doi-asserted-by":"crossref","first-page":"1438","DOI":"10.1093\/hmg\/ddr024","article-title":"Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage","volume":"20","author":"Shirendeb","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0370","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0092-8674(00)81743-2","article-title":"Nuclear inclusions in glutamine repeat disorders: are they pernicious coincidental, or beneficial?","volume":"95","author":"Sisodia","year":"1998","journal-title":"Cell"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0375","doi-asserted-by":"crossref","first-page":"354","DOI":"10.1002\/cne.20680","article-title":"Chronology of behavioral symptoms and neuropathological sequela in R6\/2 Huntington's disease transgenic mice","volume":"490","author":"Stack","year":"2005","journal-title":"J. Comp. Neurol."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0380","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","article-title":"A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes","volume":"72","year":"1993","journal-title":"Cell"},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0385","doi-asserted-by":"crossref","first-page":"8093","DOI":"10.1073\/pnas.110078997","article-title":"Nonapoptotic neurodegeneration in a transgenic mouse model of Huntington's disease","volume":"97","author":"Turmaine","year":"2000","journal-title":"Proc. Natl. Acad. Sci. U.S.A."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0390","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1016\/j.expneurol.2006.10.014","article-title":"Reduced expression of PSA-NCAM in the hippocampus and piriform cortex of the R6\/1 and R6\/2 mouse models of Huntington's disease","volume":"204","author":"van der Borght","year":"2007","journal-title":"Exp. Neurol."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0395","doi-asserted-by":"crossref","first-page":"189","DOI":"10.1016\/j.nbd.2006.12.010","article-title":"Phenotypic abnormalities in the YAC128 mouse model of Huntington disease are penetrant on multiple genetic backgrounds and modulated by strain","volume":"26","author":"Van Raamsdonk","year":"2007","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0400","doi-asserted-by":"crossref","first-page":"3823","DOI":"10.1093\/hmg\/ddi407","article-title":"Selective degeneration and nuclear localization of mutant huntingtin in the YAC128 mouse model of Huntington disease","volume":"14","author":"Van Raamsdonk","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0405","doi-asserted-by":"crossref","first-page":"365","DOI":"10.1038\/nrn1100","article-title":"Targeting programmed cell death in neurodegenerative diseases","volume":"4","author":"Vila","year":"2003","journal-title":"Nat. Rev. Neurosci."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0410","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1097\/00005072-198511000-00003","article-title":"Neuropathological classification of Huntington's disease","volume":"44","author":"Vonsattel","year":"1985","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0415","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1093\/hmg\/9.4.503","article-title":"Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice","volume":"9","author":"Wheeler","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.brainresbull.2011.07.009_bib0420","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/S0092-8674(00)80623-6","article-title":"Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease","volume":"101","author":"Yamamoto","year":"2000","journal-title":"Cell"}],"container-title":["Brain Research Bulletin"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S036192301100222X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S036192301100222X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,6]],"date-time":"2025-10-06T20:14:12Z","timestamp":1759781652000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S036192301100222X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,6]]},"references-count":84,"journal-issue":{"issue":"2-3","published-print":{"date-parts":[[2012,6]]}},"alternative-id":["S036192301100222X"],"URL":"https:\/\/doi.org\/10.1016\/j.brainresbull.2011.07.009","relation":{},"ISSN":["0361-9230"],"issn-type":[{"value":"0361-9230","type":"print"}],"subject":[],"published":{"date-parts":[[2012,6]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Light and electron microscopic characterization of the evolution of cellular pathology in the R6\/1 Huntington's disease transgenic mice","name":"articletitle","label":"Article Title"},{"value":"Brain Research Bulletin","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.brainresbull.2011.07.009","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2011 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}