{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T04:48:26Z","timestamp":1789015706179,"version":"build-2803163510"},"reference-count":191,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2023,1,1]],"date-time":"2023-01-01T00:00:00Z","timestamp":1672531200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2023,1,1]],"date-time":"2023-01-01T00:00:00Z","timestamp":1672531200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2023,8,21]],"date-time":"2023-08-21T00:00:00Z","timestamp":1692576000000},"content-version":"vor","delay-in-days":232,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["NS197397"],"award-info":[{"award-number":["NS197397"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["NS109077"],"award-info":[{"award-number":["NS109077"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["NS089750"],"award-info":[{"award-number":["NS089750"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"publisher","award":["NS089750-01A1 (MG)"],"award-info":[{"award-number":["NS089750-01A1 (MG)"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["neurotherapeuticsjournal.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neurotherapeutics"],"published-print":{"date-parts":[[2023,1]]},"DOI":"10.1007\/s13311-023-01357-5","type":"journal-article","created":{"date-parts":[[2023,4,5]],"date-time":"2023-04-05T13:13:18Z","timestamp":1680700398000},"page":"48-66","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":11,"title":["Contribution of Glial Cells to Polyglutamine Diseases: Observations from Patients and Mouse Models"],"prefix":"10.1016","volume":"20","author":[{"given":"Marija","family":"Cvetanovic","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0808-108X","authenticated-orcid":false,"given":"Michelle","family":"Gray","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1007\/s13311-023-01357-5_bib1","doi-asserted-by":"crossref","first-page":"15005","DOI":"10.1038\/nrdp.2015.5","article-title":"Huntington disease","volume":"1","author":"Bates","year":"2015","journal-title":"Nat Rev Dis Primers"},{"issue":"10","key":"10.1007\/s13311-023-01357-5_bib2","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1038\/nrn.2017.92","article-title":"Polyglutamine spinocerebellar ataxias - from genes to potential treatments","volume":"18","author":"Paulson","year":"2017","journal-title":"Nat Rev Neurosci"},{"key":"10.1007\/s13311-023-01357-5_bib3","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1146\/annurev.neuro.29.051605.113042","article-title":"Trinucleotide repeat disorders","volume":"30","author":"Orr","year":"2007","journal-title":"Annu Rev Neurosci"},{"key":"10.1007\/s13311-023-01357-5_bib4","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1016\/j.conb.2021.07.001","article-title":"Polyglutamine diseases","volume":"72","author":"Bunting","year":"2022","journal-title":"Curr Opin Neurobiol"},{"issue":"4","key":"10.1007\/s13311-023-01357-5_bib5","doi-asserted-by":"crossref","first-page":"877","DOI":"10.1016\/j.neuroscience.2004.09.053","article-title":"The neurobiology of glia in the context of water and ion homeostasis","volume":"129","author":"Simard","year":"2004","journal-title":"Neuroscience"},{"issue":"11","key":"10.1007\/s13311-023-01357-5_bib6","doi-asserted-by":"crossref","first-page":"1220","DOI":"10.2174\/13894501113149990203","article-title":"Astrocyte-neuron interaction at tripartite synapses","volume":"14","author":"P\u00e9rez-Alvarez","year":"2013","journal-title":"Curr Drug Targets"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib7","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1007\/s00401-009-0619-8","article-title":"Astrocytes: biology and pathology","volume":"119","author":"Sofroniew","year":"2010","journal-title":"Acta Neuropathol"},{"issue":"5","key":"10.1007\/s13311-023-01357-5_bib8","doi-asserted-by":"crossref","DOI":"10.1101\/cshperspect.a020388","article-title":"Astrocyte regulation of blood flow in the brain","volume":"7","author":"MacVicar","year":"2015","journal-title":"Cold Spring Harb Perspect Biol"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib9","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1152\/physrev.00042.2016","article-title":"Physiology of Astroglia","volume":"98","author":"Verkhratsky","year":"2018","journal-title":"Physiol Rev"},{"issue":"24","key":"10.1007\/s13311-023-01357-5_bib10","doi-asserted-by":"crossref","first-page":"6473","DOI":"10.1523\/JNEUROSCI.1419-07.2007","article-title":"Synaptic islands defined by the territory of a single astrocyte","volume":"27","author":"Halassa","year":"2007","journal-title":"J Neurosci"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib11","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1038\/nn.3906","article-title":"Calcium dynamics in astrocyte processes during neurovascular coupling","volume":"18","author":"Otsu","year":"2015","journal-title":"Nat Neurosci"},{"issue":"5","key":"10.1007\/s13311-023-01357-5_bib12","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1085\/jgp.201210949","article-title":"Imaging calcium microdomains within entire astrocyte territories and endfeet with GCaMPs expressed using adeno-associated viruses","volume":"141","author":"Shigetomi","year":"2013","journal-title":"J Gen Physiol"},{"issue":"6","key":"10.1007\/s13311-023-01357-5_bib13","doi-asserted-by":"crossref","first-page":"957","DOI":"10.1016\/j.immuni.2017.06.006","article-title":"Reactive astrocytes: production, function, and therapeutic potential","volume":"46","author":"Liddelow","year":"2017","journal-title":"Immunity"},{"issue":"6005","key":"10.1007\/s13311-023-01357-5_bib14","doi-asserted-by":"crossref","first-page":"841","DOI":"10.1126\/science.1194637","article-title":"Fate mapping analysis reveals that adult microglia derive from primitive macrophages","volume":"330","author":"Ginhoux","year":"2010","journal-title":"Science"},{"issue":"12","key":"10.1007\/s13311-023-01357-5_bib15","doi-asserted-by":"crossref","first-page":"1538","DOI":"10.1038\/nn2014","article-title":"Local self-renewal can sustain CNS microglia maintenance and function throughout adult life","volume":"10","author":"Ajami","year":"2007","journal-title":"Nat Neurosci"},{"issue":"4","key":"10.1007\/s13311-023-01357-5_bib16","doi-asserted-by":"crossref","first-page":"530","DOI":"10.1038\/s41593-018-0090-8","article-title":"Repopulated microglia are solely derived from the proliferation of residual microglia after acute depletion","volume":"21","author":"Huang","year":"2018","journal-title":"Nat Neurosci"},{"issue":"4","key":"10.1007\/s13311-023-01357-5_bib17","doi-asserted-by":"crossref","first-page":"225","DOI":"10.1038\/nri.2017.125","article-title":"Microglia and macrophages in brain homeostasis and disease","volume":"18","author":"Li","year":"2018","journal-title":"Nat Rev Immunol"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib18","doi-asserted-by":"crossref","first-page":"557","DOI":"10.1016\/j.celrep.2016.12.011","article-title":"Major shifts in glial regional identity are a transcriptional hallmark of human brain aging","volume":"18","author":"Soreq","year":"2017","journal-title":"Cell Rep"},{"issue":"5916","key":"10.1007\/s13311-023-01357-5_bib19","doi-asserted-by":"crossref","first-page":"390","DOI":"10.1038\/303390a0","article-title":"A glial progenitor cell that develops in vitro into an astrocyte or an oligodendrocyte depending on culture medium","volume":"303","author":"Raff","year":"1983","journal-title":"Nature"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib20","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1007\/s00401-009-0601-5","article-title":"Oligodendrocytes: biology and pathology","volume":"119","author":"Bradl","year":"2010","journal-title":"Acta Neuropathol"},{"issue":"4","key":"10.1007\/s13311-023-01357-5_bib21","doi-asserted-by":"crossref","first-page":"1061","DOI":"10.1242\/dev.111.4.1061","article-title":"Proliferation and differentiation potential of rat forebrain oligodendroglial progenitors both in vitro and in vivo","volume":"111","author":"Hardy","year":"1991","journal-title":"Development"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib22","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1038\/nn1620","article-title":"Competing waves of oligodendrocytes in the forebrain and postnatal elimination of an embryonic lineage","volume":"9","author":"Kessaris","year":"2006","journal-title":"Nat Neurosci"},{"issue":"12","key":"10.1007\/s13311-023-01357-5_bib23","doi-asserted-by":"crossref","first-page":"1392","DOI":"10.1038\/nn.2220","article-title":"PDGFRA\/NG2 glia generate myelinating oligodendrocytes and piriform projection neurons in adult mice","volume":"11","author":"Rivers","year":"2008","journal-title":"Nat Neurosci"},{"issue":"6","key":"10.1007\/s13311-023-01357-5_bib24","doi-asserted-by":"crossref","first-page":"191","DOI":"10.1016\/0962-8924(93)90213-K","article-title":"The oligodendrocyte and its many cellular processes","volume":"3","author":"Pfeiffer","year":"1993","journal-title":"Trends Cell Biol"},{"issue":"5","key":"10.1007\/s13311-023-01357-5_bib25","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1002\/1097-4547(20000901)61:5<471::AID-JNR1>3.0.CO;2-N","article-title":"NG2-expressing cells in the central nervous system: are they oligodendroglial progenitors?","volume":"61","author":"Dawson","year":"2000","journal-title":"J Neurosci Res"},{"issue":"6271","key":"10.1007\/s13311-023-01357-5_bib26","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1126\/science.aad3839","article-title":"Oligodendrocyte precursors migrate along vasculature in the developing nervous system","volume":"351","author":"Tsai","year":"2016","journal-title":"Science"},{"issue":"6","key":"10.1007\/s13311-023-01357-5_bib27","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1159\/000112359","article-title":"Transplantation of CNS fragments into the brain of shiverer mutant mice: extensive myelination by implanted oligodendrocytes. I. Immunohistochemical studies","volume":"6","author":"Lachapelle","year":"1983","journal-title":"Dev Neurosci"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib28","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1111\/j.1471-4159.1989.tb10930.x","article-title":"Differential ultrastructural localization of myelin basic protein, myelin\/oligodendroglial glycoprotein, and 2',3'-cyclic nucleotide 3'-phosphodiesterase in the CNS of adult rats","volume":"52","author":"Brunner","year":"1989","journal-title":"J Neurochem"},{"key":"10.1007\/s13311-023-01357-5_bib29","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1111\/j.1749-6632.1990.tb42378.x","article-title":"Myelin-associated glycoprotein. Location and potential functions","volume":"605","author":"Trapp","year":"1990","journal-title":"Ann N Y Acad Sci"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib30","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/S1474-4422(10)70245-3","article-title":"Huntington's disease: from molecular pathogenesis to clinical treatment","volume":"10","author":"Ross","year":"2011","journal-title":"Lancet Neurol"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib31","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1176\/jnp.15.1.109","article-title":"On chorea","volume":"15","author":"Huntington","year":"2003","journal-title":"J Neuropsychiatry Clin Neurosci"},{"issue":"6","key":"10.1007\/s13311-023-01357-5_bib32","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","author":"The Huntington's Disease Collaborative Research Group","year":"1993","journal-title":"Cell"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib33","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1016\/j.ncl.2014.09.003","article-title":"Huntington disease: pathogenesis and treatment","volume":"33","author":"Dayalu","year":"2015","journal-title":"Neurol Clin"},{"key":"10.1007\/s13311-023-01357-5_bib34","article-title":"Intermediate CAG Repeats in Huntington's disease: analysis of COHORT","volume":"2","author":"Ha","year":"2012","journal-title":"Tremor Other Hyperkinet Mov (N Y)"},{"key":"10.1007\/s13311-023-01357-5_bib35","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/B978-0-12-801893-4.00001-8","article-title":"Genetics of Huntington disease","volume":"144","author":"Nance","year":"2017","journal-title":"Handb Clin Neurol"},{"issue":"4","key":"10.1007\/s13311-023-01357-5_bib36","doi-asserted-by":"crossref","first-page":"204","DOI":"10.1038\/nrneurol.2014.24","article-title":"Huntington disease: natural history, biomarkers and prospects for therapeutics","volume":"10","author":"Ross","year":"2014","journal-title":"Nat Rev Neurol"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib37","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1111\/ene.13413","article-title":"Huntington's disease: a clinical review","volume":"25","author":"McColgan","year":"2018","journal-title":"Eur J Neurol"},{"issue":"5","key":"10.1007\/s13311-023-01357-5_bib38","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1097\/00005072-199805000-00001","article-title":"Huntington disease","volume":"57","author":"Vonsattel","year":"1998","journal-title":"J Neuropathol Exp Neurol"},{"key":"10.1007\/s13311-023-01357-5_bib39","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1016\/S0072-9752(07)01256-0","article-title":"Neuropathology of Huntington's disease","volume":"89","author":"Vonsattel","year":"2008","journal-title":"Handb Clin Neurol"},{"issue":"9","key":"10.1007\/s13311-023-01357-5_bib40","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1016\/S1474-4422(09)70170-X","article-title":"Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: cross-sectional analysis of baseline data","volume":"8","author":"Tabrizi","year":"2009","journal-title":"Lancet Neurol"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib41","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/S1474-4422(10)70276-3","article-title":"Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis","volume":"10","author":"Tabrizi","year":"2011","journal-title":"Lancet Neurol"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib42","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1016\/j.neuron.2013.05.014","article-title":"Differential innervation of direct- and indirect-pathway striatal projection neurons","volume":"79","author":"Wall","year":"2013","journal-title":"Neuron"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib43","doi-asserted-by":"crossref","first-page":"7","DOI":"10.31887\/DCNS.2016.18.1\/shaber","article-title":"Corticostriatal circuitry","volume":"18","author":"Haber","year":"2016","journal-title":"Dialogues Clin Neurosci"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib44","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1002\/ana.410370213","article-title":"Huntington's disease gene: regional and cellular expression in brain of normal and affected individuals","volume":"37","author":"Landwehrmeyer","year":"1995","journal-title":"Ann Neurol"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib45","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/j.neuron.2015.11.013","article-title":"Purification and characterization of progenitor and mature human astrocytes reveals transcriptional and functional differences with mouse","volume":"89","author":"Zhang","year":"2016","journal-title":"Neuron"},{"issue":"12","key":"10.1007\/s13311-023-01357-5_bib46","doi-asserted-by":"crossref","first-page":"1819","DOI":"10.1038\/nn.4160","article-title":"Cell type- and brain region-resolved mouse brain proteome","volume":"18","author":"Sharma","year":"2015","journal-title":"Nat Neurosci"},{"issue":"5","key":"10.1007\/s13311-023-01357-5_bib47","doi-asserted-by":"crossref","first-page":"1065","DOI":"10.1016\/0896-6273(95)90345-3","article-title":"Widespread expression of Huntington's disease gene (IT15) protein product","volume":"14","author":"Sharp","year":"1995","journal-title":"Neuron"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib48","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1006\/exnr.1998.6778","article-title":"Huntingtin protein colocalizes with lesions of neurodegenerative diseases: an investigation in Huntington's, Alzheimer's, and Pick's diseases","volume":"150","author":"Singhrao","year":"1998","journal-title":"Exp Neurol"},{"issue":"15","key":"10.1007\/s13311-023-01357-5_bib49","doi-asserted-by":"crossref","first-page":"3053","DOI":"10.1093\/hmg\/ddq212","article-title":"In vivo expression of polyglutamine-expanded huntingtin by mouse striatal astrocytes impairs glutamate transport: a correlation with Huntington's disease subjects","volume":"19","author":"Faideau","year":"2010","journal-title":"Hum Mol Genet"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib50","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1002\/glia.23050","article-title":"Frequency of nuclear mutant huntingtin inclusion formation in neurons and glia is cell-type-specific","volume":"65","author":"Jansen","year":"2017","journal-title":"Glia"},{"issue":"6","key":"10.1007\/s13311-023-01357-5_bib51","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"},{"issue":"3","key":"10.1007\/s13311-023-01357-5_bib52","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1038\/s41593-020-00783-4","article-title":"Reactive astrocyte nomenclature, definitions, and future directions","volume":"24","author":"Escartin","year":"2021","journal-title":"Nat Neurosci"},{"issue":"6","key":"10.1007\/s13311-023-01357-5_bib53","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1097\/00005072-199111000-00005","article-title":"Decreased neuronal and increased oligodendroglial densities in Huntington's disease caudate nucleus","volume":"50","author":"Myers","year":"1991","journal-title":"J Neuropathol Exp Neurol"},{"issue":"3","key":"10.1007\/s13311-023-01357-5_bib54","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1001\/archpsyc.55.3.215","article-title":"Neuronal and glial somal size in the prefrontal cortex: a postmortem morphometric study of schizophrenia and Huntington disease","volume":"55","author":"Rajkowska","year":"1998","journal-title":"Arch Gen Psychiatry"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib55","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1097\/00005072-199501000-00013","article-title":"Early loss of neostriatal striosome neurons in Huntington's disease","volume":"54","author":"Hedreen","year":"1995","journal-title":"J Neuropathol Exp Neurol"},{"issue":"12","key":"10.1007\/s13311-023-01357-5_bib56","doi-asserted-by":"crossref","first-page":"2221","DOI":"10.1002\/glia.23687","article-title":"Questions and (some) answers on reactive astrocytes","volume":"67","author":"Escartin","year":"2019","journal-title":"Glia"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib57","doi-asserted-by":"crossref","first-page":"8868","DOI":"10.1038\/s41598-018-27293-5","article-title":"Brain cell type specific gene expression and co-expression network architectures","volume":"8","author":"McKenzie","year":"2018","journal-title":"Sci Rep"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib58","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/j.neuron.2015.11.013","article-title":"Purification and characterization of progenitor and mature human astrocytes reveals transcriptional and functional differences with mouse","volume":"89","author":"Zhang","year":"2016","journal-title":"Neuron"},{"issue":"36","key":"10.1007\/s13311-023-01357-5_bib59","doi-asserted-by":"crossref","first-page":"11929","DOI":"10.1523\/JNEUROSCI.1860-14.2014","article-title":"An RNA-sequencing transcriptome and splicing database of glia, neurons, and vascular cells of the cerebral cortex","volume":"34","author":"Zhang","year":"2014","journal-title":"J Neurosci"},{"issue":"4","key":"10.1007\/s13311-023-01357-5_bib60","doi-asserted-by":"crossref","first-page":"500","DOI":"10.1038\/s41593-020-0602-1","article-title":"Astrocyte layers in the mammalian cerebral cortex revealed by a single-cell in situ transcriptomic map","volume":"23","author":"Bayraktar","year":"2020","journal-title":"Nat Neurosci"},{"issue":"5","key":"10.1007\/s13311-023-01357-5_bib61","doi-asserted-by":"crossref","first-page":"1202","DOI":"10.1016\/j.cell.2015.05.002","article-title":"Highly parallel genome-wide expression profiling of individual cells using nanoliter droplets","volume":"161","author":"Macosko","year":"2015","journal-title":"Cell"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib62","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1186\/s40478-020-0880-6","article-title":"Single-nucleus RNA-seq identifies Huntington disease astrocyte states","volume":"8","author":"Al-Dalahmah","year":"2020","journal-title":"Acta Neuropathol Commun"},{"issue":"514","key":"10.1007\/s13311-023-01357-5_bib63","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.aaw8546","article-title":"Astrocyte molecular signatures in Huntington's disease","volume":"11","author":"Diaz-Castro","year":"2019","journal-title":"Sci Transl Med"},{"issue":"18","key":"10.1007\/s13311-023-01357-5_bib64","doi-asserted-by":"crossref","first-page":"6391","DOI":"10.1523\/JNEUROSCI.6221-11.2012","article-title":"Genomic analysis of reactive astrogliosis","volume":"32","author":"Zamanian","year":"2012","journal-title":"J Neurosci"},{"issue":"7638","key":"10.1007\/s13311-023-01357-5_bib65","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1038\/nature21029","article-title":"Neurotoxic reactive astrocytes are induced by activated microglia","volume":"541","author":"Liddelow","year":"2017","journal-title":"Nature"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib66","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.neurobiolaging.2013.07.008","article-title":"Acute isolation and transcriptome characterization of cortical astrocytes and microglia from young and aged mice","volume":"35","author":"Orre","year":"2014","journal-title":"Neurobiol Aging"},{"issue":"52","key":"10.1007\/s13311-023-01357-5_bib67","doi-asserted-by":"crossref","first-page":"22480","DOI":"10.1073\/pnas.0911503106","article-title":"Expression of mutant huntingtin in mouse brain astrocytes causes age-dependent neurological symptoms","volume":"106","author":"Bradford","year":"2009","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1007\/s13311-023-01357-5_bib68","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1016\/j.nbd.2013.06.002","article-title":"Enhanced Ca-dependent glutamate release from astrocytes of the BACHD Huntington's disease mouse model","volume":"58C","author":"Lee","year":"2013","journal-title":"Neurobiol Dis"},{"key":"10.1007\/s13311-023-01357-5_bib69","doi-asserted-by":"crossref","first-page":"160","DOI":"10.1016\/j.nbd.2012.10.015","article-title":"Lack of huntingtin promotes neural stem cells differentiation into glial cells while neurons expressing huntingtin with expanded polyglutamine tracts undergo cell death","volume":"50","author":"Conforti","year":"2013","journal-title":"Neurobiol Dis"},{"issue":"3","key":"10.1007\/s13311-023-01357-5_bib70","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1385\/MN:28:3:259","article-title":"Wild-type huntingtin plays a role in brain development and neuronal survival","volume":"28","author":"Reiner","year":"2003","journal-title":"Mol Neurobiol"},{"issue":"5","key":"10.1007\/s13311-023-01357-5_bib71","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1038\/nn.3080","article-title":"An evolutionary recent neuroepithelial cell adhesion function of huntingtin implicates ADAM10-Ncadherin","volume":"15","author":"Lo Sardo","year":"2012","journal-title":"Nat Neurosci"},{"issue":"10","key":"10.1007\/s13311-023-01357-5_bib72","doi-asserted-by":"crossref","first-page":"708","DOI":"10.1038\/nrn3570","article-title":"Choosing an animal model for the study of Huntington's disease","volume":"14","author":"Pouladi","year":"2013","journal-title":"Nat Rev Neurosci"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib73","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"},{"issue":"6","key":"10.1007\/s13311-023-01357-5_bib74","doi-asserted-by":"crossref","first-page":"2193","DOI":"10.1523\/JNEUROSCI.23-06-02193.2003","article-title":"Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease","volume":"23","author":"Yu","year":"2003","journal-title":"J Neurosci"},{"issue":"6","key":"10.1007\/s13311-023-01357-5_bib75","doi-asserted-by":"crossref","first-page":"1001","DOI":"10.1083\/jcb.200508072","article-title":"Expression of mutant huntingtin in glial cells contributes to neuronal excitotoxicity","volume":"171","author":"Shin","year":"2005","journal-title":"J Cell Biol"},{"issue":"14","key":"10.1007\/s13311-023-01357-5_bib76","doi-asserted-by":"crossref","first-page":"10653","DOI":"10.1074\/jbc.M109.083287","article-title":"Mutant huntingtin in glial cells exacerbates neurological symptoms of Huntington disease mice","volume":"285","author":"Bradford","year":"2010","journal-title":"J Biol Chem"},{"issue":"11","key":"10.1007\/s13311-023-01357-5_bib77","doi-asserted-by":"crossref","first-page":"1841","DOI":"10.1002\/glia.23022","article-title":"Astrocytes are key but indirect contributors to the development of the symptomatology and pathophysiology of Huntington's disease","volume":"64","author":"Meunier","year":"2016","journal-title":"Glia"},{"key":"10.1007\/s13311-023-01357-5_bib78","article-title":"Cortical Control of striatal pathogenesis in the Cre\/LoxP conditional BAC transgenic mouse model of Huntington's disease (BACHD)","author":"Gray","year":"2008","journal-title":"Soc Neurosci. Washington, DC"},{"issue":"3","key":"10.1007\/s13311-023-01357-5_bib79","first-page":"487","article-title":"Mutant huntingtin reduction in astrocytes slows disease progression in the BACHD conditional Huntington's disease mouse model","volume":"28","author":"Wood","year":"2019","journal-title":"Hum Mol Genet"},{"key":"10.1007\/s13311-023-01357-5_bib80","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/j.mcn.2016.10.007","article-title":"Systemic application of AAV vectors targeting GFAP-expressing astrocytes in Z-Q175-KI Huntington's disease mice","volume":"77","author":"Vagner","year":"2016","journal-title":"Mol Cell Neurosci"},{"issue":"3","key":"10.1007\/s13311-023-01357-5_bib81","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1016\/j.neuron.2005.03.025","article-title":"Pathological cell-cell interactions elicited by a neuropathogenic form of mutant Huntingtin contribute to cortical pathogenesis in HD mice","volume":"46","author":"Gu","year":"2005","journal-title":"Neuron"},{"key":"10.1007\/s13311-023-01357-5_bib82","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1186\/1750-1326-2-8","article-title":"Pathological cell-cell interactions are necessary for striatal pathogenesis in a conditional mouse model of Huntington's disease","volume":"2","author":"Gu","year":"2007","journal-title":"Mol Neurodegener"},{"issue":"3","key":"10.1007\/s13311-023-01357-5_bib83","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"},{"issue":"6","key":"10.1007\/s13311-023-01357-5_bib84","doi-asserted-by":"crossref","first-page":"1146","DOI":"10.1016\/j.neuron.2020.09.021","article-title":"Context-specific striatal astrocyte molecular responses are phenotypically exploitable","volume":"108","author":"Yu","year":"2020","journal-title":"Neuron"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib85","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1016\/j.neuron.2018.03.003","article-title":"An Optical Neuron-Astrocyte Proximity Assay at Synaptic Distance Scales","volume":"98","author":"Octeau","year":"2018","journal-title":"Neuron"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib86","doi-asserted-by":"crossref","first-page":"109308","DOI":"10.1016\/j.celrep.2021.109308","article-title":"Cell-intrinsic glial pathology is conserved across human and murine models of Huntington's disease","volume":"36","author":"Benraiss","year":"2021","journal-title":"Cell Rep"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib87","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/j.stem.2018.11.010","article-title":"Human ESC-Derived Chimeric Mouse Models of Huntington's Disease Reveal Cell-Intrinsic Defects in Glial Progenitor Cell Differentiation","volume":"24","author":"Osipovitch","year":"2019","journal-title":"Cell Stem Cell"},{"issue":"25","key":"10.1007\/s13311-023-01357-5_bib88","doi-asserted-by":"crossref","first-page":"5534","DOI":"10.1523\/JNEUROSCI.2074-20.2021","article-title":"Single-nucleus RNA-Seq reveals dysregulation of striatal cell identity due to Huntington's disease mutations","volume":"41","author":"Malaiya","year":"2021","journal-title":"J Neurosci"},{"issue":"3","key":"10.1007\/s13311-023-01357-5_bib89","doi-asserted-by":"crossref","first-page":"909","DOI":"10.1046\/j.1471-4159.2000.0740909.x","article-title":"Differential effects of corticostriatal and thalamostriatal deafferentation on expression of the glutamate transporter GLT1 in the rat striatum","volume":"74","author":"Lievens","year":"2000","journal-title":"J Neurochem"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib90","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0301-0082(00)00067-8","article-title":"Glutamate uptake","volume":"65","author":"Danbolt","year":"2001","journal-title":"Prog Neurobiol"},{"issue":"4","key":"10.1007\/s13311-023-01357-5_bib91","doi-asserted-by":"crossref","first-page":"728","DOI":"10.1016\/j.neuron.2014.02.007","article-title":"Gliotransmitters travel in time and space","volume":"81","author":"Araque","year":"2014","journal-title":"Neuron"},{"issue":"10","key":"10.1007\/s13311-023-01357-5_bib92","doi-asserted-by":"crossref","first-page":"1276","DOI":"10.1038\/nn.2929","article-title":"Local Ca2+ detection and modulation of synaptic release by astrocytes","volume":"14","author":"Di Castro","year":"2011","journal-title":"Nat Neurosci"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib93","doi-asserted-by":"crossref","first-page":"413","DOI":"10.1016\/j.neuron.2014.02.041","article-title":"Conditions and constraints for astrocyte calcium signaling in the hippocampal mossy fiber pathway","volume":"82","author":"Haustein","year":"2014","journal-title":"Neuron"},{"issue":"12","key":"10.1007\/s13311-023-01357-5_bib94","doi-asserted-by":"crossref","first-page":"3453","DOI":"10.1523\/JNEUROSCI.3693-15.2016","article-title":"Dysfunctional calcium and glutamate signaling in striatal astrocytes from Huntington's disease model mice","volume":"36","author":"Jiang","year":"2016","journal-title":"J Neurosci"},{"issue":"6","key":"10.1007\/s13311-023-01357-5_bib95","doi-asserted-by":"crossref","first-page":"1170","DOI":"10.1016\/j.neuron.2018.08.015","article-title":"Reducing astrocyte calcium signaling in vivo alters striatal microcircuits and causes repetitive behavior","volume":"99","author":"Yu","year":"2018","journal-title":"Neuron"},{"issue":"7","key":"10.1007\/s13311-023-01357-5_bib96","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1016\/j.tins.2017.05.002","article-title":"Unravelling and exploiting astrocyte dysfunction in Huntington's disease","volume":"40","author":"Khakh","year":"2017","journal-title":"Trends Neurosci"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib97","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1093\/jnen\/60.2.161","article-title":"Early and progressive accumulation of reactive microglia in the Huntington disease brain","volume":"60","author":"Sapp","year":"2001","journal-title":"J Neuropathol Exp Neurol"},{"issue":"10","key":"10.1007\/s13311-023-01357-5_bib98","doi-asserted-by":"crossref","first-page":"1074","DOI":"10.1002\/glia.20526","article-title":"Ferritin accumulation in dystrophic microglia is an early event in the development of Huntington's disease","volume":"55","author":"Simmons","year":"2007","journal-title":"Glia"},{"key":"10.1007\/s13311-023-01357-5_bib99","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1016\/j.nbd.2015.08.011","article-title":"Increased central microglial activation associated with peripheral cytokine levels in premanifest Huntington's disease gene carriers","volume":"83","author":"Politis","year":"2015","journal-title":"Neurobiol Dis"},{"issue":"11","key":"10.1007\/s13311-023-01357-5_bib100","doi-asserted-by":"crossref","first-page":"1638","DOI":"10.1212\/01.wnl.0000222734.56412.17","article-title":"Microglial activation correlates with severity in Huntington disease: a clinical and PET study","volume":"66","author":"Pavese","year":"2006","journal-title":"Neurology"},{"issue":"2\u20133","key":"10.1007\/s13311-023-01357-5_bib101","doi-asserted-by":"crossref","first-page":"148","DOI":"10.1016\/j.brainresbull.2006.10.029","article-title":"Imaging microglial activation in Huntington's disease","volume":"72","author":"Tai","year":"2007","journal-title":"Brain Res Bull"},{"issue":"12","key":"10.1007\/s13311-023-01357-5_bib102","doi-asserted-by":"crossref","first-page":"1119","DOI":"10.1016\/j.bbagrm.2018.10.018","article-title":"Transcriptional regulation of translocator protein (18 kDa) (TSPO) in microglia requires Pu.1, Ap1 and Sp factors","volume":"1861","author":"Rashid","year":"2018","journal-title":"Biochim Biophys Acta Gene Regul Mech"},{"issue":"8","key":"10.1007\/s13311-023-01357-5_bib103","doi-asserted-by":"crossref","first-page":"1869","DOI":"10.1084\/jem.20080178","article-title":"A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington's disease","volume":"205","author":"Bjorkqvist","year":"2008","journal-title":"J Exp Med"},{"issue":"12","key":"10.1007\/s13311-023-01357-5_bib104","doi-asserted-by":"crossref","first-page":"1098","DOI":"10.1097\/WNR.0b013e32832e34ee","article-title":"Distinct neuroinflammatory profile in post-mortem human Huntington's disease","volume":"20","author":"Silvestroni","year":"2009","journal-title":"NeuroReport"},{"key":"10.1007\/s13311-023-01357-5_bib105","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1016\/j.bbi.2014.09.011","article-title":"Plasma inflammatory biomarkers for Huntington's disease patients and mouse model","volume":"44","author":"Chang","year":"2015","journal-title":"Brain Behav Immun"},{"key":"10.1007\/s13311-023-01357-5_bib106","doi-asserted-by":"crossref","first-page":"258","DOI":"10.3389\/fnmol.2019.00258","article-title":"The role of microglia and astrocytes in Huntington's disease","volume":"12","author":"Palpagama","year":"2019","journal-title":"Front Mol Neurosci"},{"issue":"9","key":"10.1007\/s13311-023-01357-5_bib107","doi-asserted-by":"crossref","first-page":"e0163479","DOI":"10.1371\/journal.pone.0163479","article-title":"Cerebrospinal fluid inflammatory biomarkers reflect clinical severity in Huntington's disease","volume":"11","author":"Rodrigues","year":"2016","journal-title":"PLoS ONE"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib108","doi-asserted-by":"crossref","first-page":"3473","DOI":"10.1038\/s41467-019-11441-0","article-title":"Galectin-3 is required for the microglia-mediated brain inflammation in a model of Huntington's disease","volume":"10","author":"Siew","year":"2019","journal-title":"Nat Commun"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib109","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1016\/j.nbd.2011.09.003","article-title":"Age-dependent neurovascular abnormalities and altered microglial morphology in the YAC128 mouse model of Huntington disease","volume":"45","author":"Franciosi","year":"2012","journal-title":"Neurobiol Dis"},{"issue":"12","key":"10.1007\/s13311-023-01357-5_bib110","doi-asserted-by":"crossref","first-page":"4737","DOI":"10.1172\/JCI64484","article-title":"Mutant huntingtin impairs immune cell migration in Huntington disease","volume":"122","author":"Kwan","year":"2012","journal-title":"J Clin Invest"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib111","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1523\/JNEUROSCI.4846-11.2012","article-title":"Bone marrow transplantation confers modest benefits in mouse models of Huntington's disease","volume":"32","author":"Kwan","year":"2012","journal-title":"J Neurosci"},{"issue":"4","key":"10.1007\/s13311-023-01357-5_bib112","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1038\/nn.3668","article-title":"Mutant Huntingtin promotes autonomous microglia activation via myeloid lineage-determining factors","volume":"17","author":"Crotti","year":"2014","journal-title":"Nat Neurosci"},{"issue":"10","key":"10.1007\/s13311-023-01357-5_bib113","doi-asserted-by":"crossref","first-page":"1661","DOI":"10.1093\/hmg\/ddz009","article-title":"Mutant huntingtin expression in microglia is neither required nor sufficient to cause the Huntington's disease-like phenotype in BACHD mice","volume":"28","author":"Petkau","year":"2019","journal-title":"Hum Mol Genet"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib114","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1016\/j.nbd.2011.04.015","article-title":"The role of IkappaB kinase complex in the neurobiology of Huntington's disease","volume":"43","author":"Khoshnan","year":"2011","journal-title":"Neurobiol Dis"},{"issue":"9","key":"10.1007\/s13311-023-01357-5_bib115","doi-asserted-by":"crossref","first-page":"1826","DOI":"10.1093\/hmg\/ddt036","article-title":"A critical role of astrocyte-mediated nuclear factor-kappaB-dependent inflammation in Huntington's disease","volume":"22","author":"Hsiao","year":"2013","journal-title":"Hum Mol Genet"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib116","doi-asserted-by":"crossref","first-page":"1167","DOI":"10.4049\/jimmunol.182.2.1167","article-title":"Galectin-3 deficiency reduces the severity of experimental autoimmune encephalomyelitis","volume":"182","author":"Jiang","year":"2009","journal-title":"J Immunol"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib117","doi-asserted-by":"crossref","first-page":"266","DOI":"10.1093\/brain\/awz363","article-title":"Microglial depletion prevents extracellular matrix changes and striatal volume reduction in a model of Huntington's disease","volume":"143","author":"Crapser","year":"2020","journal-title":"Brain"},{"issue":"8","key":"10.1007\/s13311-023-01357-5_bib118","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1016\/j.biopsych.2020.11.009","article-title":"Dynamics of cortical degeneration over a decade in Huntington's disease","volume":"89","author":"Johnson","year":"2021","journal-title":"Biol Psychiatry"},{"issue":"10","key":"10.1007\/s13311-023-01357-5_bib119","doi-asserted-by":"crossref","first-page":"1655","DOI":"10.1007\/s11064-007-9352-7","article-title":"Myelin breakdown and iron changes in Huntington's disease: pathogenesis and treatment implications","volume":"32","author":"Bartzokis","year":"2007","journal-title":"Neurochem Res"},{"issue":"10","key":"10.1007\/s13311-023-01357-5_bib120","doi-asserted-by":"crossref","first-page":"e109676","DOI":"10.1371\/journal.pone.0109676","article-title":"Deep white matter in Huntington's disease","volume":"9","author":"Phillips","year":"2014","journal-title":"PLoS ONE"},{"key":"10.1007\/s13311-023-01357-5_bib121","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1016\/j.cortex.2013.11.009","article-title":"White matter integrity in premanifest and early Huntington's disease is related to caudate loss and disease progression","volume":"52","author":"Novak","year":"2014","journal-title":"Cortex"},{"issue":"3","key":"10.1007\/s13311-023-01357-5_bib122","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1007\/s11682-011-9121-8","article-title":"Diffusion tensor imaging in Huntington's disease reveals distinct patterns of white matter degeneration associated with motor and cognitive deficits","volume":"5","author":"Bohanna","year":"2011","journal-title":"Brain Imaging Behav"},{"key":"10.1007\/s13311-023-01357-5_bib123","doi-asserted-by":"crossref","first-page":"236","DOI":"10.1016\/j.nicl.2018.01.029","article-title":"Complex spatial and temporally defined myelin and axonal degeneration in Huntington disease","volume":"20","author":"Rosas","year":"2018","journal-title":"NeuroImage Clin"},{"issue":"6","key":"10.1007\/s13311-023-01357-5_bib124","doi-asserted-by":"crossref","first-page":"1212","DOI":"10.1016\/j.neuron.2015.02.026","article-title":"Mutant huntingtin downregulates myelin regulatory factor-mediated myelin gene expression and affects mature oligodendrocytes","volume":"85","author":"Huang","year":"2015","journal-title":"Neuron"},{"issue":"9","key":"10.1007\/s13311-023-01357-5_bib125","doi-asserted-by":"crossref","first-page":"2508","DOI":"10.1093\/hmg\/ddv016","article-title":"Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington's disease","volume":"24","author":"Jin","year":"2015","journal-title":"Hum Mol Genet"},{"issue":"19","key":"10.1007\/s13311-023-01357-5_bib126","doi-asserted-by":"crossref","first-page":"9622","DOI":"10.1073\/pnas.1818042116","article-title":"Intrinsic mutant HTT-mediated defects in oligodendroglia cause myelination deficits and behavioral abnormalities in Huntington disease","volume":"116","author":"Ferrari Bardile","year":"2019","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"9","key":"10.1007\/s13311-023-01357-5_bib127","doi-asserted-by":"crossref","first-page":"111291","DOI":"10.1016\/j.celrep.2022.111291","article-title":"A TCF7L2-responsive suppression of both homeostatic and compensatory remyelination in Huntington disease mice","volume":"40","author":"Benraiss","year":"2022","journal-title":"Cell Rep"},{"issue":"11","key":"10.1007\/s13311-023-01357-5_bib128","doi-asserted-by":"crossref","first-page":"1000","DOI":"10.1056\/NEJMoa1104318","article-title":"Placebo-controlled trial of oral laquinimod for multiple sclerosis","volume":"366","author":"Comi","year":"2012","journal-title":"N Engl J Med"},{"issue":"21","key":"10.1007\/s13311-023-01357-5_bib129","doi-asserted-by":"crossref","DOI":"10.3390\/cells11213381","article-title":"Insights into White Matter Defect in Huntington's Disease","volume":"11","author":"Sun","year":"2022","journal-title":"Cells"},{"key":"10.1007\/s13311-023-01357-5_bib130","doi-asserted-by":"crossref","DOI":"10.12688\/f1000research.15788.1","article-title":"Recent advances in understanding dominant spinocerebellar ataxias from clinical and genetic points of view","volume":"7","author":"Coarelli","year":"2018","journal-title":"F1000Res"},{"issue":"8","key":"10.1007\/s13311-023-01357-5_bib131","doi-asserted-by":"crossref","first-page":"2749","DOI":"10.1007\/s00415-020-09815-2","article-title":"Natural history of most common spinocerebellar ataxia: a systematic review and meta-analysis","volume":"268","author":"Diallo","year":"2021","journal-title":"J Neurol"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib132","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1007\/s13311-018-00696-y","article-title":"Genetics, mechanisms, and therapeutic progress in polyglutamine spinocerebellar ataxias","volume":"16","author":"Buijsen","year":"2019","journal-title":"Neurotherapeutics"},{"key":"10.1007\/s13311-023-01357-5_bib133","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1016\/j.neulet.2018.07.005","article-title":"The cerebellum and cognition","volume":"688","author":"Schmahmann","year":"2019","journal-title":"Neurosci Lett"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib134","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s00401-012-1000-x","article-title":"Brain pathology of spinocerebellar ataxias","volume":"124","author":"Seidel","year":"2012","journal-title":"Acta Neuropathol"},{"issue":"7","key":"10.1007\/s13311-023-01357-5_bib135","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1111\/j.1365-2990.2012.01259.x","article-title":"Spinocerebellar ataxia type 1 (SCA1): new pathoanatomical and clinico-pathological insights","volume":"38","author":"Rub","year":"2012","journal-title":"Neuropathol Appl Neurobiol"},{"issue":"10","key":"10.1007\/s13311-023-01357-5_bib136","doi-asserted-by":"crossref","first-page":"3831","DOI":"10.1523\/JNEUROSCI.5612-09.2010","article-title":"Noninvasive detection of presymptomatic and progressive neurodegeneration in a mouse model of spinocerebellar ataxia type 1","volume":"30","author":"Oz","year":"2010","journal-title":"J Neurosci"},{"key":"10.1007\/s13311-023-01357-5_bib137","doi-asserted-by":"crossref","first-page":"411","DOI":"10.3389\/fneur.2020.00411","article-title":"Assessment of cerebral and cerebellar white matter microstructure in spinocerebellar ataxias 1, 2, 3, and 6 using diffusion MRI","volume":"11","author":"Park","year":"2020","journal-title":"Front Neurol"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib138","doi-asserted-by":"crossref","first-page":"fcaa184","DOI":"10.1093\/braincomms\/fcaa184","article-title":"Brainstem and striatal volume changes are detectable in under 1 year and predict motor decline in spinocerebellar ataxia type 1","volume":"2","author":"Koscik","year":"2020","journal-title":"Brain Commun"},{"issue":"4","key":"10.1007\/s13311-023-01357-5_bib139","doi-asserted-by":"crossref","first-page":"816","DOI":"10.1002\/ana.25212","article-title":"Neurochemical abnormalities in premanifest and early spinocerebellar ataxias","volume":"83","author":"Joers","year":"2018","journal-title":"Ann Neurol"},{"issue":"23","key":"10.1007\/s13311-023-01357-5_bib140","doi-asserted-by":"crossref","DOI":"10.3390\/ijms21239188","article-title":"Glia in neurodegeneration: the housekeeper, the defender and the perpetrator","volume":"21","author":"Sheeler","year":"2020","journal-title":"Int J Mol Sci"},{"issue":"9","key":"10.1007\/s13311-023-01357-5_bib141","doi-asserted-by":"crossref","first-page":"1792","DOI":"10.1016\/j.jmb.2019.01.016","article-title":"Role of microglia in ataxias","volume":"431","author":"Ferro","year":"2019","journal-title":"J Mol Biol"},{"issue":"3","key":"10.1007\/s13311-023-01357-5_bib142","doi-asserted-by":"crossref","first-page":"452","DOI":"10.1007\/s12311-021-01311-1","article-title":"Consensus paper: strengths and weaknesses of animal models of spinocerebellar ataxias and their clinical implications","volume":"21","author":"Cendelin","year":"2022","journal-title":"Cerebellum"},{"issue":"6","key":"10.1007\/s13311-023-01357-5_bib143","doi-asserted-by":"crossref","first-page":"905","DOI":"10.1016\/S0896-6273(02)00733-X","article-title":"A long CAG repeat in the mouse Sca1 locus replicates SCA1 features and reveals the impact of protein solubility on selective neurodegeneration","volume":"34","author":"Watase","year":"2002","journal-title":"Neuron"},{"issue":"7650","key":"10.1007\/s13311-023-01357-5_bib144","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1038\/nature22044","article-title":"Antisense oligonucleotide therapy for spinocerebellar ataxia type 2","volume":"544","author":"Scoles","year":"2017","journal-title":"Nature"},{"issue":"19","key":"10.1007\/s13311-023-01357-5_bib145","doi-asserted-by":"crossref","first-page":"7385","DOI":"10.1523\/JNEUROSCI.17-19-07385.1997","article-title":"Purkinje cell expression of a mutant allele of SCA1 in transgenic mice leads to disparate effects on motor behaviors, followed by a progressive cerebellar dysfunction and histological alterations","volume":"17","author":"Clark","year":"1997","journal-title":"J Neurosci"},{"issue":"8","key":"10.1007\/s13311-023-01357-5_bib146","doi-asserted-by":"crossref","first-page":"1540","DOI":"10.1002\/jnr.23984","article-title":"Genetically modified rodent models of SCA17","volume":"95","author":"Cui","year":"2017","journal-title":"J Neurosci Res"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib147","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1002\/ana.25264","article-title":"Oligonucleotide therapy mitigates disease in spinocerebellar ataxia type 3 mice","volume":"84","author":"McLoughlin","year":"2018","journal-title":"Ann Neurol"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib148","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1016\/j.cell.2013.05.059","article-title":"Second cistron in CACNA1A gene encodes a transcription factor mediating cerebellar development and SCA6","volume":"154","author":"Du","year":"2013","journal-title":"Cell"},{"issue":"8","key":"10.1007\/s13311-023-01357-5_bib149","doi-asserted-by":"crossref","first-page":"1604","DOI":"10.1523\/JNEUROSCI.1954-20.2021","article-title":"Impaired oligodendrocyte maturation is an early feature in SCA3 disease pathogenesis","volume":"42","author":"Schuster","year":"2022","journal-title":"J Neurosci"},{"key":"10.1007\/s13311-023-01357-5_bib150","doi-asserted-by":"crossref","first-page":"4","DOI":"10.1186\/2053-8871-1-4","article-title":"From mice to men: lessons from mutant ataxic mice","volume":"1","author":"Cendelin","year":"2014","journal-title":"Cerebellum Ataxias"},{"issue":"5","key":"10.1007\/s13311-023-01357-5_bib151","doi-asserted-by":"crossref","first-page":"918","DOI":"10.1016\/j.neuron.2019.04.021","article-title":"Universal transform or multiple functionality? Understanding the contribution of the human cerebellum across task domains","volume":"102","author":"Diedrichsen","year":"2019","journal-title":"Neuron"},{"issue":"5518","key":"10.1007\/s13311-023-01357-5_bib152","doi-asserted-by":"crossref","first-page":"926","DOI":"10.1126\/science.1058827","article-title":"Glia-synapse interaction through Ca2+-permeable AMPA receptors in Bergmann glia","volume":"292","author":"Iino","year":"2001","journal-title":"Science"},{"issue":"20","key":"10.1007\/s13311-023-01357-5_bib153","doi-asserted-by":"crossref","first-page":"7911","DOI":"10.1073\/pnas.1120380109","article-title":"Bergmann glia modulate cerebellar Purkinje cell bistability via Ca2+-dependent K+ uptake","volume":"109","author":"Wang","year":"2012","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib154","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1080\/14734220600724569","article-title":"Interactions between Purkinje neurones and Bergmann glia","volume":"5","author":"Bellamy","year":"2006","journal-title":"Cerebellum"},{"issue":"10","key":"10.1007\/s13311-023-01357-5_bib155","doi-asserted-by":"crossref","first-page":"1302","DOI":"10.1038\/nn1750","article-title":"Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport","volume":"9","author":"Custer","year":"2006","journal-title":"Nat Neurosci"},{"issue":"15","key":"10.1007\/s13311-023-01357-5_bib156","doi-asserted-by":"crossref","DOI":"10.3390\/ijms22157822","article-title":"Protective effect of memantine on Bergmann glia and purkinje cells morphology in optogenetic model of neurodegeneration in mice","volume":"22","author":"Shuvaev","year":"2021","journal-title":"Int J Mol Sci"},{"issue":"3","key":"10.1007\/s13311-023-01357-5_bib157","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1016\/S0896-6273(00)80086-0","article-title":"Knockout of glutamate transporters reveals a major role for astroglial transport in excitotoxicity and clearance of glutamate","volume":"16","author":"Rothstein","year":"1996","journal-title":"Neuron"},{"key":"10.1007\/s13311-023-01357-5_bib158","doi-asserted-by":"crossref","first-page":"998408","DOI":"10.3389\/fncel.2022.998408","article-title":"Single nuclei RNA sequencing investigation of the Purkinje cell and glial changes in the cerebellum of transgenic spinocerebellar ataxia type 1 mice","volume":"16","author":"Borgenheimer","year":"2022","journal-title":"Front Cell Neurosci"},{"key":"10.1007\/s13311-023-01357-5_bib159","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1016\/j.neuroscience.2015.01.003","article-title":"Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1","volume":"289","author":"Cvetanovic","year":"2015","journal-title":"Neuroscience"},{"issue":"9","key":"10.1007\/s13311-023-01357-5_bib160","doi-asserted-by":"crossref","first-page":"1972","DOI":"10.1002\/glia.23451","article-title":"Astroglia contribute to the pathogenesis of spinocerebellar ataxia type 1 (SCA1) in a biphasic, stage-of-disease specific manner","volume":"66","author":"Kim","year":"2018","journal-title":"Glia"},{"key":"10.1007\/s13311-023-01357-5_bib161","article-title":"Differential effects of Wnt-\u03b2-catenin signaling in Purkinje cells and Bergmann glia in SCA1","author":"Luttik","year":"2021","journal-title":"bioRxiv"},{"key":"10.1007\/s13311-023-01357-5_bib162","article-title":"Reactive Bergmann glia play a central role in Spinocerebellar ataxia inflammation via the JNK pathway","author":"Edamakanti","year":"2022","journal-title":"bioRxiv"},{"key":"10.1007\/s13311-023-01357-5_bib163","doi-asserted-by":"crossref","first-page":"509","DOI":"10.3389\/fncel.2018.00509","article-title":"Brain derived neurotrophic factor (BDNF) delays onset of pathogenesis in transgenic mouse model of spinocerebellar ataxia type 1 (SCA1)","volume":"12","author":"Mellesmoen","year":"2018","journal-title":"Front Cell Neurosci"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib164","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1007\/s12311-014-0605-0","article-title":"Decreased expression of glutamate transporter GLAST in Bergmann glia is associated with the loss of Purkinje neurons in the spinocerebellar ataxia type 1","volume":"14","author":"Cvetanovic","year":"2015","journal-title":"Cerebellum"},{"key":"10.1007\/s13311-023-01357-5_bib165","article-title":"Longitudinal single-cell transcriptional dynamics throughout neurodegeneration in SCA1","author":"Tejwani","year":"2021","journal-title":"bioRxiv"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib166","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1186\/s12974-017-0880-z","article-title":"Inhibition of colony-stimulating factor 1 receptor early in disease ameliorates motor deficits in SCA1 mice","volume":"14","author":"Qu","year":"2017","journal-title":"J Neuroinflammation"},{"issue":"7","key":"10.1007\/s13311-023-01357-5_bib167","doi-asserted-by":"crossref","first-page":"e0200013","DOI":"10.1371\/journal.pone.0200013","article-title":"Inhibition of NF-kappaB signaling in IKKbetaF\/F;LysM Cre mice causes motor deficits but does not alter pathogenesis of Spinocerebellar ataxia type 1","volume":"13","author":"Ferro","year":"2018","journal-title":"PLoS ONE"},{"issue":"20","key":"10.1007\/s13311-023-01357-5_bib168","doi-asserted-by":"crossref","first-page":"4015","DOI":"10.1007\/s00018-020-03520-z","article-title":"Pathogenic mechanisms underlying spinocerebellar ataxia type 1","volume":"77","author":"Tejwani","year":"2020","journal-title":"Cell Mol Life Sci"},{"issue":"12","key":"10.1007\/s13311-023-01357-5_bib169","doi-asserted-by":"crossref","first-page":"1482","DOI":"10.1038\/s41587-019-0336-3","article-title":"Visualizing structure and transitions in high-dimensional biological data","volume":"37","author":"Moon","year":"2019","journal-title":"Nat Biotechnol"},{"key":"10.1007\/s13311-023-01357-5_bib170","article-title":"Spatial and temporal diversity of astrocyte phenotypes in Spinocerebellar ataxia type 1 mice","author":"Rosa","year":"2022","journal-title":"bioRxiv"},{"issue":"4","key":"10.1007\/s13311-023-01357-5_bib171","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1007\/s10048-021-00662-5","article-title":"Spinocerebellar ataxias (SCAs) caused by common mutations","volume":"22","author":"Muller","year":"2021","journal-title":"Neurogenetics"},{"issue":"2","key":"10.1007\/s13311-023-01357-5_bib172","doi-asserted-by":"crossref","first-page":"533","DOI":"10.1007\/s00415-018-9076-4","article-title":"Spinocerebellar ataxia: an update","volume":"266","author":"Sullivan","year":"2019","journal-title":"J Neurol"},{"issue":"4","key":"10.1007\/s13311-023-01357-5_bib173","doi-asserted-by":"crossref","first-page":"667","DOI":"10.1016\/j.neuron.2015.10.038","article-title":"RAN translation in Huntington disease","volume":"88","author":"Banez-Coronel","year":"2015","journal-title":"Neuron"},{"issue":"16","key":"10.1007\/s13311-023-01357-5_bib174","doi-asserted-by":"crossref","first-page":"4653","DOI":"10.1113\/JP271897","article-title":"Cellular and circuit mechanisms underlying spinocerebellar ataxias","volume":"594","author":"Meera","year":"2016","journal-title":"J Physiol"},{"key":"10.1007\/s13311-023-01357-5_bib175","doi-asserted-by":"crossref","first-page":"41","DOI":"10.1016\/j.neulet.2018.02.005","article-title":"Ion channel dysfunction in cerebellar ataxia","volume":"688","author":"Bushart","year":"2019","journal-title":"Neurosci Lett"},{"key":"10.1007\/s13311-023-01357-5_bib176","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.semcdb.2015.03.010","article-title":"Disturbed calcium signaling in spinocerebellar ataxias and Alzheimer's disease","volume":"40","author":"Egorova","year":"2015","journal-title":"Semin Cell Dev Biol"},{"issue":"10","key":"10.1007\/s13311-023-01357-5_bib177","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1016\/j.tins.2006.08.004","article-title":"Astrocytic complexity distinguishes the human brain","volume":"29","author":"Oberheim","year":"2006","journal-title":"Trends Neurosci"},{"issue":"10","key":"10.1007\/s13311-023-01357-5_bib178","doi-asserted-by":"crossref","first-page":"3276","DOI":"10.1523\/JNEUROSCI.4707-08.2009","article-title":"Uniquely hominid features of adult human astrocytes","volume":"29","author":"Oberheim","year":"2009","journal-title":"J Neurosci"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib179","doi-asserted-by":"crossref","first-page":"3958","DOI":"10.1038\/s41467-021-24232-3","article-title":"Conservation and divergence of vulnerability and responses to stressors between human and mouse astrocytes","volume":"12","author":"Li","year":"2021","journal-title":"Nat Commun"},{"issue":"5","key":"10.1007\/s13311-023-01357-5_bib180","doi-asserted-by":"crossref","first-page":"779","DOI":"10.1093\/hmg\/9.5.779","article-title":"Repeat instability and motor incoordination in mice with a targeted expanded CAG repeat in the Sca1 locus","volume":"9","author":"Lorenzetti","year":"2000","journal-title":"Hum Mol Genet"},{"issue":"3","key":"10.1007\/s13311-023-01357-5_bib181","doi-asserted-by":"crossref","first-page":"504","DOI":"10.1038\/nn.4222","article-title":"Microglial brain region-dependent diversity and selective regional sensitivities to aging","volume":"19","author":"Grabert","year":"2016","journal-title":"Nat Neurosci"},{"issue":"12","key":"10.1007\/s13311-023-01357-5_bib182","doi-asserted-by":"crossref","first-page":"3321","DOI":"10.1016\/j.neurobiolaging.2015.08.024","article-title":"Aging causes morphological alterations in astrocytes and microglia in human substantia nigra pars compacta","volume":"36","author":"Jyothi","year":"2015","journal-title":"Neurobiol Aging"},{"issue":"9","key":"10.1007\/s13311-023-01357-5_bib183","doi-asserted-by":"crossref","first-page":"e45250","DOI":"10.1371\/journal.pone.0045250","article-title":"The neuron-astrocyte-microglia triad in normal brain ageing and in a model of neuroinflammation in the rat hippocampus","volume":"7","author":"Cerbai","year":"2012","journal-title":"PLoS ONE"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib184","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1016\/j.celrep.2017.12.039","article-title":"The aging astrocyte transcriptome from multiple regions of the mouse brain","volume":"22","author":"Boisvert","year":"2018","journal-title":"Cell Rep"},{"issue":"8","key":"10.1007\/s13311-023-01357-5_bib185","doi-asserted-by":"crossref","first-page":"E1896","DOI":"10.1073\/pnas.1800165115","article-title":"Normal aging induces A1-like astrocyte reactivity","volume":"115","author":"Clarke","year":"2018","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"8","key":"10.1007\/s13311-023-01357-5_bib186","doi-asserted-by":"crossref","first-page":"726","DOI":"10.1038\/s43587-022-00257-1","article-title":"A distinct astrocyte subtype in the aging mouse brain characterized by impaired protein homeostasis","volume":"2","author":"Lee","year":"2022","journal-title":"Nature Aging"},{"issue":"3","key":"10.1007\/s13311-023-01357-5_bib187","doi-asserted-by":"crossref","first-page":"NS20210008","DOI":"10.1042\/NS20210008","article-title":"Oligodendrocytes in the aging brain","volume":"5","author":"Sams","year":"2021","journal-title":"Neuronal Signal"},{"issue":"3","key":"10.1007\/s13311-023-01357-5_bib188","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1016\/j.neuron.2018.12.020","article-title":"Oligodendrocyte progenitor cells become regionally diverse and heterogeneous with age","volume":"101","author":"Spitzer","year":"2019","journal-title":"Neuron"},{"issue":"6","key":"10.1007\/s13311-023-01357-5_bib189","doi-asserted-by":"crossref","first-page":"937","DOI":"10.1016\/0092-8674(95)90273-2","article-title":"SCA1 transgenic mice: a model for neurodegeneration caused by an expanded CAG trinucleotide repeat","volume":"82","author":"Burright","year":"1995","journal-title":"Cell"},{"issue":"1","key":"10.1007\/s13311-023-01357-5_bib190","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1016\/S0896-6273(00)80764-3","article-title":"A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration","volume":"23","author":"Hodgson","year":"1999","journal-title":"Neuron"},{"issue":"12","key":"10.1007\/s13311-023-01357-5_bib191","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0049838","article-title":"Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington's disease: zQ175","volume":"7","author":"Menalled","year":"2012","journal-title":"PLoS One"}],"container-title":["Neurotherapeutics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s13311-023-01357-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s13311-023-01357-5\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1878747923001058?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1878747923001058?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s13311-023-01357-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T06:33:19Z","timestamp":1761805999000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1878747923001058"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,1]]},"references-count":191,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2023,1]]}},"alternative-id":["S1878747923001058"],"URL":"https:\/\/doi.org\/10.1007\/s13311-023-01357-5","relation":{},"ISSN":["1878-7479"],"issn-type":[{"value":"1878-7479","type":"print"}],"subject":[],"published":{"date-parts":[[2023,1]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Contribution of Glial Cells to Polyglutamine Diseases: Observations from Patients and Mouse Models","name":"articletitle","label":"Article Title"},{"value":"Neurotherapeutics","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1007\/s13311-023-01357-5","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2023 The American Society for Experimental NeuroTherapeutics, Inc. Published by Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}