{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T02:07:37Z","timestamp":1791857257520,"version":"4.3.4"},"reference-count":95,"publisher":"Elsevier BV","issue":"5","license":[{"start":{"date-parts":[[2020,9,1]],"date-time":"2020-09-01T00:00:00Z","timestamp":1598918400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,9,1]],"date-time":"2020-09-01T00:00:00Z","timestamp":1598918400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2021,9,9]],"date-time":"2021-09-09T00:00:00Z","timestamp":1631145600000},"content-version":"vor","delay-in-days":373,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100007457","name":"JPB Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007457","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100005725","name":"CHDI Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005725","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["cell.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neuron"],"published-print":{"date-parts":[[2020,9]]},"DOI":"10.1016\/j.neuron.2020.06.021","type":"journal-article","created":{"date-parts":[[2020,7,17]],"date-time":"2020-07-17T10:35:55Z","timestamp":1594982155000},"page":"891-908.e8","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":280,"title":["Cell Type-Specific Transcriptomics Reveals that Mutant Huntingtin Leads to Mitochondrial RNA Release and Neuronal Innate Immune Activation"],"prefix":"10.1016","volume":"107","author":[{"given":"Hyeseung","family":"Lee","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6299-9612","authenticated-orcid":false,"given":"Robert J.","family":"Fenster","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S. Sebastian","family":"Pineda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Whitney S.","family":"Gibbs","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8734-2326","authenticated-orcid":false,"given":"Shahin","family":"Mohammadi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jose","family":"Davila-Velderrain","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francisco J.","family":"Garcia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martine","family":"Therrien","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hailey S.","family":"Novis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fan","family":"Gao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0401-9329","authenticated-orcid":false,"given":"Hilary","family":"Wilkinson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"Vogt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manolis","family":"Kellis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2583-1578","authenticated-orcid":false,"given":"Matthew J.","family":"LaVoie","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Myriam","family":"Heiman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.neuron.2020.06.021_bib1","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1002\/ana.410310412","article-title":"Preferential loss of striato-external pallidal projection neurons in presymptomatic Huntington\u2019s disease","volume":"31","author":"Albin","year":"1992","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.neuron.2020.06.021_bib2","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1186\/1750-1172-8-83","article-title":"Biotin-responsive basal ganglia disease should be renamed biotin-thiamine-responsive basal ganglia disease: a retrospective review of the clinical, radiological and molecular findings of 18 new cases","volume":"8","author":"Alfadhel","year":"2013","journal-title":"Orphanet J. Rare Dis."},{"key":"10.1016\/j.neuron.2020.06.021_bib3","doi-asserted-by":"crossref","first-page":"838","DOI":"10.1038\/ng.3593","article-title":"Functional characterization of somatic mutations in cancer using network-based inference of protein activity","volume":"48","author":"Alvarez","year":"2016","journal-title":"Nat. Genet."},{"key":"10.1016\/j.neuron.2020.06.021_bib4","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":"Ba\u00f1ez-Coronel","year":"2015","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2020.06.021_bib5","article-title":"Dimensionality reduction for visualizing single-cell data using UMAP","author":"Becht","year":"2018","journal-title":"Nat. Biotechnol."},{"key":"10.1016\/j.neuron.2020.06.021_bib6","doi-asserted-by":"crossref","first-page":"3065","DOI":"10.1093\/hmg\/ddi340","article-title":"Contribution of nuclear and extranuclear polyQ to neurological phenotypes in mouse models of Huntington\u2019s disease","volume":"14","author":"Benn","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.neuron.2020.06.021_bib7","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1016\/S0091-679X(01)65006-4","article-title":"Assaying mitochondrial respiratory complex activity in mitochondria isolated from human cells and tissues","volume":"65","author":"Birch-Machin","year":"2001","journal-title":"Methods Cell Biol."},{"key":"10.1016\/j.neuron.2020.06.021_bib8","doi-asserted-by":"crossref","first-page":"853","DOI":"10.1016\/0306-4522(88)90073-5","article-title":"Cellular substrate of the histochemically defined striosome\/matrix system of the caudate nucleus: a combined Golgi and immunocytochemical study in cat and ferret","volume":"24","author":"Bolam","year":"1988","journal-title":"Neuroscience"},{"key":"10.1016\/j.neuron.2020.06.021_bib9","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. USA"},{"key":"10.1016\/j.neuron.2020.06.021_bib10","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1021\/acschemneuro.6b00333","article-title":"The striosome and matrix compartments of the striatum: a path through the labyrinth from neurochemistry toward function","volume":"8","author":"Brimblecombe","year":"2017","journal-title":"ACS Chem. Neurosci."},{"key":"10.1016\/j.neuron.2020.06.021_bib11","doi-asserted-by":"crossref","first-page":"253","DOI":"10.1016\/j.pneurobio.2006.11.001","article-title":"The corticostriatal pathway in Huntington\u2019s disease","volume":"81","author":"Cepeda","year":"2007","journal-title":"Prog. Neurobiol."},{"key":"10.1016\/j.neuron.2020.06.021_bib12","doi-asserted-by":"crossref","first-page":"1187","DOI":"10.1002\/j.1460-2075.1995.tb07102.x","article-title":"RAR-specific agonist\/antagonists which dissociate transactivation and AP1 transrepression inhibit anchorage-independent cell proliferation","volume":"14","author":"Chen","year":"1995","journal-title":"EMBO J."},{"key":"10.1016\/j.neuron.2020.06.021_bib13","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1186\/1471-2105-14-128","article-title":"Enrichr: interactive and collaborative HTML5 gene list enrichment analysis tool","volume":"14","author":"Chen","year":"2013","journal-title":"BMC Bioinformatics"},{"key":"10.1016\/j.neuron.2020.06.021_bib14","doi-asserted-by":"crossref","first-page":"51","DOI":"10.1038\/s41467-017-02495-z","article-title":"C9ORF72 GGGGCC repeat-associated non-AUG translation is upregulated by stress through eIF2\u03b1 phosphorylation","volume":"9","author":"Cheng","year":"2018","journal-title":"Nat. Commun."},{"key":"10.1016\/j.neuron.2020.06.021_bib15","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1016\/j.cell.2017.12.038","article-title":"Human ADAR1 prevents endogenous RNA from triggering translational shutdown","volume":"172","author":"Chung","year":"2018","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2020.06.021_bib16","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1016\/S0165-0173(00)00039-4","article-title":"Chemical anatomy of striatal interneurons in normal individuals and in patients with Huntington\u2019s disease","volume":"34","author":"Cicchetti","year":"2000","journal-title":"Brain Res. Brain Res. Rev."},{"key":"10.1016\/j.neuron.2020.06.021_bib17","doi-asserted-by":"crossref","first-page":"887","DOI":"10.1016\/j.cell.2019.06.036","article-title":"CAG repeat not polyglutamine length determines timing of Huntington\u2019s disease onset","volume":"178","year":"2019","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2020.06.021_bib18","doi-asserted-by":"crossref","first-page":"2334","DOI":"10.1042\/bj0292334","article-title":"The differential effect of radium radiation on the carbohydrate metabolism of normal and tumour tissues irradiated at low temperature","volume":"29","author":"Crabtree","year":"1935","journal-title":"Biochem. J."},{"key":"10.1016\/j.neuron.2020.06.021_bib19","doi-asserted-by":"crossref","first-page":"429","DOI":"10.1038\/nri3850","article-title":"Aicardi-Gouti\u00e8res syndrome and the type I interferonopathies","volume":"15","author":"Crow","year":"2015","journal-title":"Nat. Rev. Immunol."},{"key":"10.1016\/j.neuron.2020.06.021_bib20","doi-asserted-by":"crossref","first-page":"1421","DOI":"10.1111\/j.1471-4159.2010.07046.x","article-title":"Interferon-\u03b2 induces apoptosis in human SH-SY5Y neuroblastoma cells through activation of JAK-STAT signaling and down-regulation of PI3K\/Akt pathway","volume":"115","author":"Dedoni","year":"2010","journal-title":"J.\u00a0Neurochem."},{"key":"10.1016\/j.neuron.2020.06.021_bib21","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1038\/s41586-018-0363-0","article-title":"Mitochondrial double-stranded RNA triggers antiviral signalling in humans","volume":"560","author":"Dhir","year":"2018","journal-title":"Nature"},{"key":"10.1016\/j.neuron.2020.06.021_bib22","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1093\/bioinformatics\/bts635","article-title":"STAR: ultrafast universal RNA-seq aligner","volume":"29","author":"Dobin","year":"2013","journal-title":"Bioinformatics"},{"key":"10.1016\/j.neuron.2020.06.021_bib23","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1016\/j.cell.2008.10.029","article-title":"Application of a translational profiling approach for the comparative analysis of CNS cell types","volume":"135","author":"Doyle","year":"2008","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2020.06.021_bib24","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/j.nbd.2008.09.014","article-title":"Intergenerational and striatal CAG repeat instability in Huntington\u2019s disease knock-in mice involve different DNA repair genes","volume":"33","author":"Dragileva","year":"2009","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.neuron.2020.06.021_bib25","doi-asserted-by":"crossref","first-page":"763","DOI":"10.1038\/nmeth.2996","article-title":"Targeting cells with single vectors using multiple-feature Boolean logic","volume":"11","author":"Fenno","year":"2014","journal-title":"Nat. Methods"},{"key":"10.1016\/j.neuron.2020.06.021_bib26","doi-asserted-by":"crossref","first-page":"1590","DOI":"10.1002\/jnr.24493","article-title":"Phenotype onset in Huntington\u2019s disease knock-in mice is correlated with the incomplete splicing of the mutant huntingtin gene","volume":"97","author":"Franich","year":"2019","journal-title":"J.\u00a0Neurosci. Res."},{"key":"10.1016\/j.neuron.2020.06.021_bib27","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1146\/annurev.ne.15.030192.001441","article-title":"The neostriatal mosaic: multiple levels of compartmental organization in the basal ganglia","volume":"15","author":"Gerfen","year":"1992","journal-title":"Annu. Rev. Neurosci."},{"key":"10.1016\/j.neuron.2020.06.021_bib28","doi-asserted-by":"crossref","first-page":"877","DOI":"10.1038\/sj.embor.7400235","article-title":"Rational design of RAR-selective ligands revealed by RARbeta crystal stucture","volume":"5","author":"Germain","year":"2004","journal-title":"EMBO Rep."},{"key":"10.1016\/j.neuron.2020.06.021_bib29","doi-asserted-by":"crossref","first-page":"3467","DOI":"10.1073\/pnas.0800048105","article-title":"DNA instability in postmitotic neurons","volume":"105","author":"Gonitel","year":"2008","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2020.06.021_bib30","doi-asserted-by":"crossref","first-page":"5723","DOI":"10.1073\/pnas.75.11.5723","article-title":"Histochemically distinct compartments in the striatum of human, monkeys, and cat demonstrated by acetylthiocholinesterase staining","volume":"75","author":"Graybiel","year":"1978","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2020.06.021_bib31","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\u2019s disease chromosomes","volume":"72","year":"1993","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2020.06.021_bib32","doi-asserted-by":"crossref","first-page":"2688","DOI":"10.1016\/j.celrep.2017.11.018","article-title":"Control of Huntington\u2019s disease-associated phenotypes by the striatum-enriched transcription factor Foxp2","volume":"21","author":"Hachigian","year":"2017","journal-title":"Cell Rep."},{"key":"10.1016\/j.neuron.2020.06.021_bib33","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1038\/nbt.4091","article-title":"Batch effects in single-cell RNA-sequencing data are corrected by matching mutual nearest neighbors","volume":"36","author":"Haghverdi","year":"2018","journal-title":"Nat. Biotechnol."},{"key":"10.1016\/j.neuron.2020.06.021_bib34","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1097\/00005072-199501000-00013","article-title":"Early loss of neostriatal striosome neurons in Huntington\u2019s disease","volume":"54","author":"Hedreen","year":"1995","journal-title":"J.\u00a0Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.neuron.2020.06.021_bib35","doi-asserted-by":"crossref","first-page":"e50717","DOI":"10.1371\/journal.pone.0050717","article-title":"Characterization of neurophysiological and behavioral changes, MRI brain volumetry and 1H MRS in zQ175 knock-in mouse model of Huntington\u2019s disease","volume":"7","author":"Heikkinen","year":"2012","journal-title":"PLoS ONE"},{"key":"10.1016\/j.neuron.2020.06.021_bib36","doi-asserted-by":"crossref","first-page":"738","DOI":"10.1016\/j.cell.2008.10.028","article-title":"A translational profiling approach for the molecular characterization of CNS cell types","volume":"135","author":"Heiman","year":"2008","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2020.06.021_bib37","doi-asserted-by":"crossref","first-page":"1282","DOI":"10.1038\/nprot.2014.085","article-title":"Cell type-specific mRNA purification by translating ribosome affinity purification (TRAP)","volume":"9","author":"Heiman","year":"2014","journal-title":"Nat. Protoc."},{"key":"10.1016\/j.neuron.2020.06.021_bib38","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1038\/s41587-019-0113-3","article-title":"Efficient integration of heterogeneous single-cell transcriptomes using Scanorama","volume":"37","author":"Hie","year":"2019","journal-title":"Nat. Biotechnol."},{"key":"10.1016\/j.neuron.2020.06.021_bib39","doi-asserted-by":"crossref","first-page":"965","DOI":"10.1093\/hmg\/ddl013","article-title":"Regional and cellular gene expression changes in human Huntington\u2019s disease brain","volume":"15","author":"Hodges","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.neuron.2020.06.021_bib40","doi-asserted-by":"crossref","first-page":"4473","DOI":"10.1016\/j.febslet.2007.08.022","article-title":"The oxindole\/imidazole derivative C16 reduces in\u00a0vivo brain PKR activation","volume":"581","author":"Ingrand","year":"2007","journal-title":"FEBS Lett."},{"issue":"W1","key":"10.1016\/j.neuron.2020.06.021_bib41","doi-asserted-by":"crossref","first-page":"W212","DOI":"10.1093\/nar\/gkz446","article-title":"ChEA3: transcription factor enrichment analysis by orthogonal omics integration","volume":"47","author":"Keenan","year":"2019","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.neuron.2020.06.021_bib42","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1016\/j.tins.2017.05.002","article-title":"Unravelling and exploiting astrocyte dysfunction in Huntington\u2019s disease","volume":"40","author":"Khakh","year":"2017","journal-title":"Trends Neurosci."},{"key":"10.1016\/j.neuron.2020.06.021_bib43","doi-asserted-by":"crossref","first-page":"1051","DOI":"10.1016\/j.molcel.2018.07.029","article-title":"PKR senses nuclear and mitochondrial signals by interacting with endogenous double-stranded RNAs","volume":"71","author":"Kim","year":"2018","journal-title":"Mol. Cell"},{"key":"10.1016\/j.neuron.2020.06.021_bib44","first-page":"1668","article-title":"Thiamine deficiency in infants: MR findings in the brain","volume":"26","author":"Kornreich","year":"2005","journal-title":"AJNR Am. J. Neuroradiol."},{"key":"10.1016\/j.neuron.2020.06.021_bib45","doi-asserted-by":"crossref","first-page":"1289","DOI":"10.1038\/s41592-019-0619-0","article-title":"Fast, sensitive, and accurate integration of single cell data with Harmony","volume":"16","author":"Korsunsky","year":"2019","journal-title":"Nat. Methods"},{"key":"10.1016\/j.neuron.2020.06.021_bib46","article-title":"Presto scales Wilcoxon and auROC analyses to millions of observations","author":"Korsunsky","year":"2019","journal-title":"bioRxiv"},{"issue":"W1","key":"10.1016\/j.neuron.2020.06.021_bib47","doi-asserted-by":"crossref","first-page":"W90","DOI":"10.1093\/nar\/gkw377","article-title":"Enrichr: a comprehensive gene set enrichment analysis web server 2016 update","volume":"44","author":"Kuleshov","year":"2016","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.neuron.2020.06.021_bib48","doi-asserted-by":"crossref","first-page":"2438","DOI":"10.1093\/bioinformatics\/btq466","article-title":"ChEA: transcription factor regulation inferred from integrating genome-wide ChIP-X experiments","volume":"26","author":"Lachmann","year":"2010","journal-title":"Bioinformatics"},{"key":"10.1016\/j.neuron.2020.06.021_bib49","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1186\/1471-2105-9-559","article-title":"WGCNA: an R package for weighted correlation network analysis","volume":"9","author":"Langfelder","year":"2008","journal-title":"BMC Bioinformatics"},{"key":"10.1016\/j.neuron.2020.06.021_bib50","doi-asserted-by":"crossref","first-page":"e61505","DOI":"10.1371\/journal.pone.0061505","article-title":"When is hub gene selection better than standard meta-analysis?","volume":"8","author":"Langfelder","year":"2013","journal-title":"PLoS ONE"},{"key":"10.1016\/j.neuron.2020.06.021_bib51","doi-asserted-by":"crossref","first-page":"623","DOI":"10.1038\/nn.4256","article-title":"Integrated genomics and proteomics define huntingtin CAG length-dependent networks in mice","volume":"19","author":"Langfelder","year":"2016","journal-title":"Nat. Neurosci."},{"key":"10.1016\/j.neuron.2020.06.021_bib52","doi-asserted-by":"crossref","first-page":"1754","DOI":"10.1093\/bioinformatics\/btp324","article-title":"Fast and accurate short read alignment with Burrows-Wheeler transform","volume":"25","author":"Li","year":"2009","journal-title":"Bioinformatics"},{"key":"10.1016\/j.neuron.2020.06.021_bib53","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"},{"key":"10.1016\/j.neuron.2020.06.021_bib54","doi-asserted-by":"crossref","first-page":"994","DOI":"10.1038\/cr.2010.103","article-title":"Tom70 mediates activation of interferon regulatory factor 3 on mitochondria","volume":"20","author":"Liu","year":"2010","journal-title":"Cell Res."},{"key":"10.1016\/j.neuron.2020.06.021_bib55","doi-asserted-by":"crossref","first-page":"550","DOI":"10.1186\/s13059-014-0550-8","article-title":"Moderated estimation of fold change and dispersion for RNA-seq data with DESeq2","volume":"15","author":"Love","year":"2014","journal-title":"Genome Biol."},{"key":"10.1016\/j.neuron.2020.06.021_bib56","doi-asserted-by":"crossref","first-page":"1259","DOI":"10.1093\/hmg\/9.9.1259","article-title":"Decreased expression of striatal signaling genes in a mouse model of Huntington\u2019s disease","volume":"9","author":"Luthi-Carter","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.neuron.2020.06.021_bib57","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.neuron.2020.06.021_bib58","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1038\/70598","article-title":"Msh2 deficiency prevents in\u00a0vivo somatic instability of the CAG repeat in Huntington disease transgenic mice","volume":"23","author":"Manley","year":"1999","journal-title":"Nat. Genet."},{"key":"10.1016\/j.neuron.2020.06.021_bib59","doi-asserted-by":"crossref","first-page":"4320","DOI":"10.1016\/j.celrep.2019.11.096","article-title":"A spatiomolecular map of the striatum","volume":"29","author":"M\u00e4rtin","year":"2019","journal-title":"Cell Rep."},{"key":"10.1016\/j.neuron.2020.06.021_bib60","doi-asserted-by":"crossref","first-page":"332","DOI":"10.1038\/s41586-019-1195-2","article-title":"Single-cell transcriptomic analysis of Alzheimer\u2019s disease","volume":"570","author":"Mathys","year":"2019","journal-title":"Nature"},{"key":"10.1016\/j.neuron.2020.06.021_bib61","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\u2019s disease knock-in mice","volume":"22","author":"Menalled","year":"2002","journal-title":"J.\u00a0Neurosci."},{"key":"10.1016\/j.neuron.2020.06.021_bib62","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1002\/cne.10776","article-title":"Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington\u2019s disease with 140 CAG repeats","volume":"465","author":"Menalled","year":"2003","journal-title":"J.\u00a0Comp. Neurol."},{"key":"10.1016\/j.neuron.2020.06.021_bib63","doi-asserted-by":"crossref","first-page":"e49838","DOI":"10.1371\/journal.pone.0049838","article-title":"Comprehensive behavioral and molecular characterization of a new knock-in mouse model of Huntington\u2019s disease: zQ175","volume":"7","author":"Menalled","year":"2012","journal-title":"PLoS ONE"},{"key":"10.1016\/j.neuron.2020.06.021_bib64","doi-asserted-by":"crossref","first-page":"5805","DOI":"10.1128\/jvi.66.10.5805-5814.1992","article-title":"Constitutive expression of human double-stranded RNA-activated p68 kinase in murine cells mediates phosphorylation of eukaryotic initiation factor 2 and partial resistance to encephalomyocarditis virus growth","volume":"66","author":"Meurs","year":"1992","journal-title":"J.\u00a0Virol."},{"key":"10.1016\/j.neuron.2020.06.021_bib65","doi-asserted-by":"crossref","first-page":"4439","DOI":"10.1093\/emboj\/19.17.4439","article-title":"Recruitment of human muscleblind proteins to (CUG)(n) expansions associated with myotonic dystrophy","volume":"19","author":"Miller","year":"2000","journal-title":"EMBO J."},{"key":"10.1016\/j.neuron.2020.06.021_bib66","doi-asserted-by":"crossref","first-page":"1516","DOI":"10.1038\/s41467-018-03933-2","article-title":"A geometric approach to characterize the functional identity of single cells","volume":"9","author":"Mohammadi","year":"2018","journal-title":"Nat. Commun."},{"key":"10.1016\/j.neuron.2020.06.021_bib67","article-title":"A multiresolution framework to characterize single-state cell landscapes","author":"Mohammadi","year":"2019","journal-title":"bioRxiv"},{"key":"10.1016\/j.neuron.2020.06.021_bib68","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1038\/nrn2151","article-title":"Neuronal subtype specification in the cerebral cortex","volume":"8","author":"Molyneaux","year":"2007","journal-title":"Nat. Rev. Neurosci."},{"key":"10.1016\/j.neuron.2020.06.021_bib69","doi-asserted-by":"crossref","first-page":"5389","DOI":"10.1172\/JCI70317","article-title":"Circadian clock proteins regulate neuronal redox homeostasis and neurodegeneration","volume":"123","author":"Musiek","year":"2013","journal-title":"J.\u00a0Clin. Invest."},{"key":"10.1016\/j.neuron.2020.06.021_bib70","doi-asserted-by":"crossref","first-page":"3859","DOI":"10.1007\/s12035-016-0010-4","article-title":"Genome-wide analysis of RAR\u03b2 transcriptional targets in mouse striatum links retinoic acid signaling with Huntington\u2019s disease and other neurodegenerative disorders","volume":"54","author":"Niewiadomska-Cimicka","year":"2017","journal-title":"Mol. Neurobiol."},{"key":"10.1016\/j.neuron.2020.06.021_bib71","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1016\/S0169-328X(03)00341-3","article-title":"Coexpression of junctophilin type 3 and type 4 in brain","volume":"118","author":"Nishi","year":"2003","journal-title":"Brain Res. Mol. Brain Res."},{"key":"10.1016\/j.neuron.2020.06.021_bib72","doi-asserted-by":"crossref","first-page":"5733","DOI":"10.1073\/pnas.85.15.5733","article-title":"Differential loss of striatal projection neurons in Huntington disease","volume":"85","author":"Reiner","year":"1988","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2020.06.021_bib73","doi-asserted-by":"crossref","first-page":"e47","DOI":"10.1093\/nar\/gkv007","article-title":"limma powers differential expression analyses for RNA-sequencing and microarray studies","volume":"43","author":"Ritchie","year":"2015","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.neuron.2020.06.021_bib74","doi-asserted-by":"crossref","first-page":"1563","DOI":"10.1016\/j.cell.2014.11.037","article-title":"Apoptotic caspases prevent the induction of type I interferons by mitochondrial DNA","volume":"159","author":"Rongvaux","year":"2014","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2020.06.021_bib75","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.neuron.2020.06.021_bib76","doi-asserted-by":"crossref","first-page":"1015","DOI":"10.1016\/j.cell.2018.07.028","article-title":"Molecular diversity and specializations among the cells of the adult mouse brain","volume":"174","author":"Saunders","year":"2018","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2020.06.021_bib77","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1016\/j.nbd.2008.09.016","article-title":"Voxel-based morphometry in the R6\/2 transgenic mouse reveals differences between genotypes not seen with manual 2D morphometry","volume":"33","author":"Sawiak","year":"2009","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.neuron.2020.06.021_bib78","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1083\/jcb.201607019","article-title":"CLUH regulates mitochondrial metabolism by controlling translation and decay of target mRNAs","volume":"216","author":"Schatton","year":"2017","journal-title":"J.\u00a0Cell Biol."},{"key":"10.1016\/j.neuron.2020.06.021_bib79","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.nbd.2013.05.007","article-title":"The mitochondrial disease associated protein Ndufaf2 is dispensable for Complex-1 assembly but critical for the regulation of oxidative stress","volume":"58","author":"Schlehe","year":"2013","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.neuron.2020.06.021_bib80","doi-asserted-by":"crossref","first-page":"4593","DOI":"10.1038\/s41467-018-07053-9","article-title":"Tissue-resident memory T\u00a0cells populate the human brain","volume":"9","author":"Smolders","year":"2018","journal-title":"Nat. Commun."},{"key":"10.1016\/j.neuron.2020.06.021_bib81","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1038\/s41398-018-0281-9","article-title":"Convergence of independent DISC1 mutations on impaired neurite growth via decreased UNC5D expression","volume":"8","author":"Srikanth","year":"2018","journal-title":"Transl. Psychiatry"},{"key":"10.1016\/j.neuron.2020.06.021_bib82","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1097\/00005072-198511000-00003","article-title":"Neuropathological classification of Huntington\u2019s disease","volume":"44","author":"Vonsattel","year":"1985","journal-title":"J.\u00a0Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.neuron.2020.06.021_bib83","doi-asserted-by":"crossref","first-page":"2380","DOI":"10.1111\/j.1471-4159.2007.04978.x","article-title":"Activation of double-stranded RNA-activated protein kinase by mild impairment of oxidative metabolism in neurons","volume":"103","author":"Wang","year":"2007","journal-title":"J.\u00a0Neurochem."},{"key":"10.1016\/j.neuron.2020.06.021_bib84","doi-asserted-by":"crossref","first-page":"16923","DOI":"10.1038\/srep16923","article-title":"Efficient test and visualization of multi-set intersections","volume":"5","author":"Wang","year":"2015","journal-title":"Sci. Rep."},{"key":"10.1016\/j.neuron.2020.06.021_bib85","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/j.neuron.2020.01.004","article-title":"Genome-wide in\u00a0vivo CNS screening identifies genes that modify CNS neuronal survival and mHTT toxicity","volume":"106","author":"Wertz","year":"2020","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2020.06.021_bib86","doi-asserted-by":"crossref","first-page":"553","DOI":"10.1038\/nature14156","article-title":"Mitochondrial DNA stress primes the antiviral innate immune response","volume":"520","author":"West","year":"2015","journal-title":"Nature"},{"key":"10.1016\/j.neuron.2020.06.021_bib87","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1093\/hmg\/8.1.115","article-title":"Length-dependent gametic CAG repeat instability in the Huntington\u2019s disease knock-in mouse","volume":"8","author":"Wheeler","year":"1999","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.neuron.2020.06.021_bib88","doi-asserted-by":"crossref","first-page":"1549","DOI":"10.1016\/j.cell.2014.11.036","article-title":"Apoptotic caspases suppress mtDNA-induced STING-mediated type I IFN production","volume":"159","author":"White","year":"2014","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2020.06.021_bib89","doi-asserted-by":"crossref","first-page":"1195","DOI":"10.1073\/pnas.1814092116","article-title":"The harmonic mean p-value for combining dependent tests","volume":"116","author":"Wilson","year":"2019","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.neuron.2020.06.021_bib90","doi-asserted-by":"crossref","first-page":"1772","DOI":"10.1046\/j.1471-4159.1994.63051772.x","article-title":"3-nitropropionic acid toxicity in the striatum","volume":"63","author":"W\u00fcllner","year":"1994","journal-title":"J.\u00a0Neurochem."},{"key":"10.1016\/j.neuron.2020.06.021_bib91","doi-asserted-by":"crossref","first-page":"999","DOI":"10.1016\/j.cell.2018.06.021","article-title":"Molecular architecture of the mouse nervous system","volume":"174","author":"Zeisel","year":"2018","journal-title":"Cell"},{"key":"10.1016\/j.neuron.2020.06.021_bib92","doi-asserted-by":"crossref","first-page":"Article17","DOI":"10.2202\/1544-6115.1128","article-title":"A general framework for weighted gene co-expression network analysis","volume":"4","author":"Zhang","year":"2005","journal-title":"Stat. Appl. Genet. Mol. Biol."},{"key":"10.1016\/j.neuron.2020.06.021_bib93","doi-asserted-by":"crossref","first-page":"R137","DOI":"10.1186\/gb-2008-9-9-r137","article-title":"Model-based analysis of ChIP-seq (MACS)","volume":"9","author":"Zhang","year":"2008","journal-title":"Genome Biol."},{"key":"10.1016\/j.neuron.2020.06.021_bib94","doi-asserted-by":"crossref","first-page":"785","DOI":"10.1016\/j.neuron.2013.05.029","article-title":"Rapid single-step induction of functional neurons from human pluripotent stem cells","volume":"78","author":"Zhang","year":"2013","journal-title":"Neuron"},{"key":"10.1016\/j.neuron.2020.06.021_bib95","doi-asserted-by":"crossref","first-page":"905","DOI":"10.1152\/physrev.00041.2009","article-title":"Molecular mechanisms and potential therapeutical targets in Huntington\u2019s disease","volume":"90","author":"Zuccato","year":"2010","journal-title":"Physiol. Rev."}],"container-title":["Neuron"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S089662732030475X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S089662732030475X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T07:26:43Z","timestamp":1760254003000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S089662732030475X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,9]]},"references-count":95,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2020,9]]}},"alternative-id":["S089662732030475X"],"URL":"https:\/\/doi.org\/10.1016\/j.neuron.2020.06.021","relation":{"has-review":[{"id-type":"doi","id":"10.3410\/f.738342597.793578347","asserted-by":"object"}]},"ISSN":["0896-6273"],"issn-type":[{"value":"0896-6273","type":"print"}],"subject":[],"published":{"date-parts":[[2020,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Cell Type-Specific Transcriptomics Reveals that Mutant Huntingtin Leads to Mitochondrial RNA Release and Neuronal Innate Immune Activation","name":"articletitle","label":"Article Title"},{"value":"Neuron","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.neuron.2020.06.021","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2020 Elsevier Inc.","name":"copyright","label":"Copyright"}]}}