{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T09:02:05Z","timestamp":1792746125759,"version":"4.5.0"},"reference-count":71,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,10,21]],"date-time":"2024-10-21T00:00:00Z","timestamp":1729468800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2024,10,21]],"date-time":"2024-10-21T00:00:00Z","timestamp":1729468800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["108890\/Z\/15\/Z"],"award-info":[{"award-number":["108890\/Z\/15\/Z"]}],"id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000381","name":"Multiple Sclerosis Society","doi-asserted-by":"publisher","award":["Centre"],"award-info":[{"award-number":["Centre"]}],"id":[{"id":"10.13039\/501100000381","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100017510","name":"UK Dementia Research Institute","doi-asserted-by":"publisher","award":["x"],"award-info":[{"award-number":["x"]}],"id":[{"id":"10.13039\/501100017510","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"german research foundation","doi-asserted-by":"crossref","award":["CRC\/TRR167"],"award-info":[{"award-number":["CRC\/TRR167"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["acta neuropathol commun"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Huntington\u2019s disease (HD) is an autosomal dominant neurodegenerative disease with a fatal outcome. There is accumulating evidence of a prominent role of glia in the pathology of HD, and we investigated this by conducting single nuclear RNA sequencing (snRNAseq) of human post mortem brain in four differentially affected regions; caudate nucleus, frontal cortex, hippocampus and cerebellum. Across 127,205 nuclei from donors with HD and age\/sex matched controls, we found heterogeneity of glia which is altered in HD. We describe prominent changes in the abundance of certain subtypes of astrocytes, microglia, oligodendrocyte precursor cells and oligodendrocytes between HD and control samples, and these differences are widespread across brain regions. Furthermore, we highlight possible mechanisms that characterise the glial contribution to HD pathology including depletion of myelinating oligodendrocytes, an oligodendrocyte-specific upregulation of the calmodulin-dependent 3\u2019,5\u2019-cyclic nucleotide phosphodiesterase 1\u00a0A (\n                    <jats:italic>PDE1A<\/jats:italic>\n                    ) and an upregulation of molecular chaperones as a cross-glial signature and a potential adaptive response to the accumulation of mutant huntingtin (mHTT). Our results support the hypothesis that glia have an important role in the pathology of HD, and show that all types of glia are affected in the disease.\n                  <\/jats:p>","DOI":"10.1186\/s40478-024-01871-3","type":"journal-article","created":{"date-parts":[[2024,10,20]],"date-time":"2024-10-20T22:01:59Z","timestamp":1729461719000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Mapping the glial transcriptome in Huntington\u2019s disease using snRNAseq: selective disruption of glial signatures across brain regions"],"prefix":"10.1186","volume":"12","author":[{"given":"Sunniva M. K.","family":"B\u00f8strand","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luise A.","family":"Seeker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadine","family":"Bestard-Cuche","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nina-Lydia","family":"Kazakou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"J\u00e4kel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Boyd","family":"Kenkhuis","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Neil C.","family":"Henderson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanne T.","family":"de Bot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Willeke M. C.","family":"van Roon-Mom","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Josef","family":"Priller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Williams","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2024,10,21]]},"reference":[{"issue":"9557","key":"1871_CR1","doi-asserted-by":"publisher","first-page":"218","DOI":"10.1016\/S0140-6736(07)60111-1","volume":"369","author":"FO Walker","year":"2007","unstructured":"Walker FO (2007) Huntington\u2019s disease. Lancet 369(9557):218\u2013228","journal-title":"Lancet"},{"issue":"2","key":"1871_CR2","doi-asserted-by":"publisher","first-page":"255","DOI":"10.1602\/neurorx.1.2.255","volume":"1","author":"RH Myers","year":"2004","unstructured":"Myers RH (2004) Huntington\u2019s disease genetics. NeuroRx 1(2):255\u2013262","journal-title":"NeuroRx"},{"issue":"1","key":"1871_CR3","doi-asserted-by":"publisher","first-page":"50","DOI":"10.1002\/glia.23050","volume":"65","author":"AHP Jansen","year":"2017","unstructured":"Jansen AHP, Hal M, Kelder IC, Meier RT, Ruiter AA, Schut MH, Smith DL, Grit C, Brouwer N, Kamphuis W, Boddeke HWGM, Dunnen WFA, Roon WMC, Bates GP, Hol EM, Reits EA (2017) Frequency of nuclear mutant huntingtin inclusion formation in neurons and glia is cell-type-specific. Glia 65(1):50\u201361. https:\/\/doi.org\/10.1002\/glia.23050","journal-title":"Glia"},{"issue":"2","key":"1871_CR4","doi-asserted-by":"publisher","first-page":"258","DOI":"10.1002\/hbm.21008","volume":"32","author":"M Politis","year":"2011","unstructured":"Politis M, Pavese N, Tai YF, Kiferle L, Mason SL, Brooks DJ, Tabrizi SJ, Barker RA, Piccini P (2011) Microglial activation in regions related to cognitive function predicts disease onset in Huntington\u2019s disease: a multimodal imaging study. Hum Brain Mapp 32(2):258\u2013270","journal-title":"Hum Brain Mapp"},{"key":"1871_CR5","doi-asserted-by":"publisher","first-page":"33","DOI":"10.1016\/j.coph.2015.09.008","volume":"26","author":"R Andre","year":"2016","unstructured":"Andre R, Carty L, Tabrizi SJ (2016) Disruption of immune cell function by mutant huntingtin in Huntington\u2019s disease pathogenesis. Curr Opin Pharmacol 26:33\u201338. https:\/\/doi.org\/10.1016\/j.coph.2015.09.008","journal-title":"Curr Opin Pharmacol"},{"issue":"8","key":"1871_CR6","doi-asserted-by":"publisher","first-page":"1302","DOI":"10.1002\/mds.28109","volume":"35","author":"C Casella","year":"2020","unstructured":"Casella C, Lipp I, Rosser A, Jones DK, Metzler-Baddeley C (2020) A critical review of white matter changes in Huntington\u2019s disease. Mov Disord 35(8):1302\u20131311","journal-title":"Mov Disord"},{"issue":"9","key":"1871_CR7","doi-asserted-by":"publisher","first-page":"3516","DOI":"10.1002\/hbm.24191","volume":"39","author":"S Gregory","year":"2018","unstructured":"Gregory S, Crawford H, Seunarine K, Leavitt B, Durr A, Roos RAC, Scahill RI, Tabrizi SJ, Rees G, Langbehn D, Orth M (2018) Natural biological variation of white matter microstructure is accentuated in Huntington\u2019s disease. Hum Brain Mapp 39(9):3516\u20133527. https:\/\/doi.org\/10.1002\/hbm","journal-title":"Hum Brain Mapp"},{"key":"1871_CR8","doi-asserted-by":"publisher","unstructured":"Phillips OR, Joshi SH, Squitieri F, Sanchez-Castaneda C, Narr K, Shattuck DW, Caltagirone C, Sabatini U, Di Paola M (2016) Major superficial white matter abnormalities in Huntington\u2019s disease. Front NeuroSci 10. https:\/\/doi.org\/10.3389\/fnins.2016.00197","DOI":"10.3389\/fnins.2016.00197"},{"issue":"1","key":"1871_CR9","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1016\/j.pscychresns.2005.05.011","volume":"140","author":"SAJ Reading","year":"2005","unstructured":"Reading SAJ, Yassa MA, Bakker A, Dziorny AC, Gourley LM, Yallapragada V, Rosenblatt A, Margolis RL, Aylward EH, Brandt J, Mori S, Van Zijl P, Bassett SS, Ross CA (2005) Regional white matter change in pre-symptomatic Huntington\u2019s disease: a diffusion tensor imaging study. Psychiatry Res - Neuroimaging 140(1):55\u201362.https:\/\/doi.org\/10.1016\/j.pscychresns.2005.05.011","journal-title":"Psychiatry Res - Neuroimaging"},{"issue":"5","key":"1871_CR10","doi-asserted-by":"publisher","first-page":"369","DOI":"10.1097\/00005072-199805000-00001","volume":"57","author":"JPG Vonsattel","year":"1998","unstructured":"Vonsattel JPG, Difiglia M (1998) Huntington Disease. J Neuropathology Experimental Neurol 57(5):369\u2013384. https:\/\/doi.org\/10.1097\/00005072-199805000-00001. https:\/\/academic.oup.com\/jnen\/articlepdf\/57\/5\/369\/8276375\/57-5-369.pdf","journal-title":"J Neuropathology Experimental Neurol"},{"issue":"4688","key":"1871_CR11","doi-asserted-by":"publisher","first-page":"770","DOI":"10.1126\/science.3155875","volume":"227","author":"GA Graveland","year":"1985","unstructured":"Graveland GA, Williams RS, DiFiglia M (1985) Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington\u2019s disease. Science 227(4688):770\u2013773. https:\/\/doi.org\/10.1126\/science.3155875. https:\/\/www.science.org\/doi\/pdf\/10.1126\/science.3155875","journal-title":"Science"},{"issue":"3","key":"1871_CR12","doi-asserted-by":"publisher","first-page":"171","DOI":"10.1007\/s11682-011-9121-8","volume":"5","author":"I Bohanna","year":"2011","unstructured":"Bohanna I, Georgiou-Karistianis N, Sritharan A, Asadi H, Johnston L, Churchyard A, Egan G (2011) Diffusion Tensor Imaging in Huntington\u2019s disease reveals distinct patterns of white matter degeneration associated with motor and cognitive deficits. Brain Imaging Behav 5(3):171\u2013180. https:\/\/doi.org\/10.1007\/s11682-011-9121-8","journal-title":"Brain Imaging Behav"},{"key":"1871_CR13","doi-asserted-by":"publisher","first-page":"236","DOI":"10.1016\/j.nicl.2018.01.029","volume":"20","author":"H Rosas","year":"2018","unstructured":"Rosas H, Wilkens P, Salat D, Mercaldo N, Vangel M, Yendiki A, Hersch S (2018) Complex spatial and temporally defined myelin and axonal degeneration in Huntington disease. NeuroImage: Clin 20:236\u2013242","journal-title":"NeuroImage: Clin"},{"issue":"3","key":"1871_CR14","doi-asserted-by":"publisher","first-page":"239","DOI":"10.3233\/JHD-150160","volume":"4","author":"S Gregory","year":"2015","unstructured":"Gregory S, Scahill RI, Seunarine KK, Stopford C, Zhang H, Zhang J, Orth M, Durr A, Roos RAC, Langbehn DR, Long JD, Johnson H, Rees G, Tabrizi SJ, Craufurd D, Investigators T-H (2015) Neuropsychiatry and White Matter Microstructure in Huntington\u2019s Disease. J Huntington\u2019s Disease 4(3):239\u2013249. https:\/\/doi.org\/10.3233\/JHD-150160","journal-title":"J Huntington\u2019s Disease"},{"issue":"15","key":"1871_CR15","doi-asserted-by":"publisher","first-page":"1208","DOI":"10.1212\/WNL.0b013e3181d8c20a","volume":"74","author":"D Stoffers","year":"2010","unstructured":"Stoffers D, Sheldon S, Kuperman JM, Goldstein J, Corey-Bloom J, Aron AR (2010) Contrasting gray and white matter changes in preclinical Huntington disease: an MRI study. Neurology 74(15):1208\u20131216. https:\/\/doi.org\/10.1212\/WNL.0b013e3181d8c20a","journal-title":"Neurology"},{"key":"1871_CR16","doi-asserted-by":"publisher","first-page":"450","DOI":"10.1016\/j.nicl.2016.02.014","volume":"11","author":"AV Faria","year":"2016","unstructured":"Faria AV, Ratnanather JT, Tward DJ, Lee DS, Van Den Noort F, Wu D, Brown T, Johnson H, Paulsen JS, Ross CA, Younes L, Miller MI (2016) Linking white matter and deep gray matter alterations in Premanifest Huntington disease. NeuroImage: Clin 11:450\u2013460. https:\/\/doi.org\/10.1016\/j.nicl","journal-title":"NeuroImage: Clin"},{"key":"1871_CR17","doi-asserted-by":"publisher","unstructured":"McColgan P, Seunarine KK, Gregory S, Razi A, Papoutsi M, Long JD, Mills JA, Johnson E, Durr A, Roos RA, Leavitt BR, Stout JC, Scahill RI, Clark CA, Rees G, Tabrizi SJ, Investigators TTOH (2017) Topological length of white matter connections predicts their rate of atrophy in premanifest Huntington\u2019s disease. JCI Insight 2(8). https:\/\/doi.org\/10.1172\/jci.insight","DOI":"10.1172\/jci.insight"},{"issue":"2","key":"1871_CR18","doi-asserted-by":"publisher","first-page":"106","DOI":"10.1002\/acn3.384","volume":"4","author":"P McColgan","year":"2017","unstructured":"McColgan P, Gregory S, Razi A, Seunarine KK, Gargouri F, Durr A, Roos RAC, Leavitt BR, Scahill RI, Clark CA, Tabrizi SJ, Rees G, Coleman A, Decolongon J, Fan M, Petkau T, Jauffret C, Justo D, Lehericy S, Nigaud K, Valabr`egue R, Choonderbeek A, Hart EPT, Moss H, Crawford DJ, Johnson H, Papoutsi E, Berna M, Reilmann C, Weber R, Stout N, Labuschagne J, Landwehrmeyer I, Orth B, Johnson M (2017) White matter predicts functional connectivity in Premanifest Huntington\u2019s disease. Ann Clin Transl Neurol 4(2):106\u2013118. https:\/\/doi.org\/10.1002\/acn3.384","journal-title":"Ann Clin Transl Neurol"},{"issue":"13","key":"1871_CR19","doi-asserted-by":"publisher","first-page":"2621","DOI":"10.1093\/hmg\/ddw122","volume":"25","author":"RT Yi Teo","year":"2016","unstructured":"Yi Teo RT, Hong X, Yu-Taeger L, Huang Y, Tan LJ, Xie Y, To XV, Guo L, Rajendran R, Novati A, Calaminus C, Riess O, Hayden MR, Nguyen HP, Chuang KH, Pouladi MA (2016) Structural and molecular myelination deficits occur prior to neuronal loss in the YAC128 and BACHD models of Huntington disease. Hum Mol Genet 25(13):2621\u20132632. https:\/\/doi.org\/10.1093\/hmg\/ddw122","journal-title":"Hum Mol Genet"},{"key":"1871_CR20","doi-asserted-by":"publisher","unstructured":"Ferrari Bardile C, Garcia-Miralles M, Caron NS, Rayan NA, Langley SR, Harmston N, Rondelli AM, Teo RTY, Waltl S, Anderson LM, Bae H-G, Jung S, Williams A, Prabhakar S, Petretto E, Hayden MR, Pouladi MA (2019) Intrinsic mutant HTT-mediated defects in oligodendroglia cause myelination deficits and behavioral abnormalities in Huntington disease. Proc Natl Acad Sci 116(19):201818042. https:\/\/doi.org\/10.1073\/pnas.1818042116","DOI":"10.1073\/pnas.1818042116"},{"issue":"6","key":"1871_CR21","doi-asserted-by":"publisher","first-page":"1212","DOI":"10.1016\/j.neuron.2015.02.026","volume":"85","author":"B Huang","year":"2015","unstructured":"Huang B, Wei WJ, Wang G, Gaertig MA, Feng Y, Wang W, Li XJ, Li S (2015) Mutant huntingtin downregulates myelin regulatory factor-mediated myelin gene expression and affects mature oligodendrocytes. Neuron 85(6):1212\u20131226. https:\/\/doi.org\/10.1016\/j.neuron.2015.02.026","journal-title":"Neuron"},{"issue":"1","key":"1871_CR22","doi-asserted-by":"publisher","first-page":"107","DOI":"10.1016\/j.stem.2018.11.010","volume":"24","author":"M Osipovitch","year":"2019","unstructured":"Osipovitch M, Asenjo Martinez A, Mariani JN, Cornwell A, Dhaliwal S, Zou L, Chandler-Militello D, Wang S, Li X, Benraiss S-J, Agate R, Lampp A, Benraiss A, Windrem MS, Goldman SA (2019) Human esc-derived chimeric mouse models of Huntington\u2019s disease reveal cell-intrinsic defects in glial progenitor cell differentiation. Cell Stem Cell 24(1):107\u20131227. https:\/\/doi.org\/10.1016\/j.stem.2018.11.010","journal-title":"Cell Stem Cell"},{"issue":"1","key":"1871_CR23","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1038\/ncomms11758","volume":"7","author":"A Benraiss","year":"2016","unstructured":"Benraiss A, Wang S, Herrlinger S, Li X, Chandler-Militello D, Mauceri J, Burm HB, Toner M, Osipovitch M, Xu J (2016) Human glia can both induce and rescue aspects of disease phenotype in Huntington disease. Nat Commun 7(1):1\u201313","journal-title":"Nat Commun"},{"issue":"52","key":"1871_CR24","doi-asserted-by":"publisher","first-page":"22480","DOI":"10.1073\/pnas.0911503106","volume":"106","author":"J Bradford","year":"2009","unstructured":"Bradford J, Shin J-Y, Roberts M, Wang C-E, Li X-J, Li S (2009) Expression of mutant huntingtin in mouse brain astrocytes causes agedependent neurological symptoms. Proc Natl Acad Sci 106(52):22480\u201322485. https:\/\/doi.org\/10.1073\/pnas.0911503106. https:\/\/www.pnas.org\/doi\/pdf\/10.1073\/pnas.0911503106","journal-title":"Proc Natl Acad Sci"},{"issue":"3","key":"1871_CR25","first-page":"487","volume":"28","author":"TE Wood","year":"2019","unstructured":"Wood TE, Barry J, Yang Z, Cepeda C, Levine MS, Gray M (2019) Mutant huntingtin reduction in astrocytes slows disease progression in the bachd conditional Huntington\u2019s disease mouse model. Hum Mol Genet 28(3):487\u2013500","journal-title":"Hum Mol Genet"},{"issue":"10","key":"1871_CR26","doi-asserted-by":"publisher","first-page":"1661","DOI":"10.1093\/hmg\/ddz009","volume":"28","author":"TL Petkau","year":"2019","unstructured":"Petkau TL, Hill A, Connolly C, Lu G, Wagner P, Kosior N, Blanco J, Leavitt BR (2019) Mutant huntingtin expression in microglia is neither required nor sufficient to cause the Huntington\u2019s disease-like phenotype in BACHD mice. Hum Mol Genet 28(10):1661\u20131670. https:\/\/doi.org\/10.1093\/hmg\/ddz009","journal-title":"Hum Mol Genet"},{"issue":"8","key":"1871_CR27","doi-asserted-by":"publisher","first-page":"1869","DOI":"10.1084\/jem.20080178","volume":"205","author":"M Bj\u00f6rkqvist","year":"2008","unstructured":"Bj\u00f6rkqvist M, Wild EJ, Thiele J, Silvestroni A, Andre R, Lahiri N, Raibon E, Lee RV, Benn CL, Soulet D et al (2008) A novel pathogenic pathway of immune activation detectable before clinical onset in Huntington\u2019s disease. J Exp Med 205(8):1869\u20131877","journal-title":"J Exp Med"},{"issue":"11","key":"1871_CR28","doi-asserted-by":"publisher","first-page":"2866","DOI":"10.1038\/s41591-023-02566-3","volume":"29","author":"DK Wilton","year":"2023","unstructured":"Wilton DK, Mastro K, Heller MD, Gergits FW, Willing CR, Fahey JB, Frouin A, Daggett A, Gu X, Kim YA et al (2023) Microglia and complement mediate early corticostriatal synapse loss and cognitive dysfunction in Huntington\u2019s disease. Nat Med 29(11):2866\u20132884","journal-title":"Nat Med"},{"issue":"1","key":"1871_CR29","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/s40478-023-01568-z","volume":"11","author":"LA Seeker","year":"2023","unstructured":"Seeker LA, Bestard-Cuche N, J\u00e4kel S, Kazakou N-L, B\u00f8strand SM, Wagstaff LJ, Cholewa-Waclaw J, Kilpatrick AM, Van Bruggen D, Kabbe M et al (2023) Brain matters: unveiling the distinct contributions of region, age, and sex to glia diversity and CNS function. Acta Neuropathol Commun 11(1):1\u201323","journal-title":"Acta Neuropathol Commun"},{"issue":"1","key":"1871_CR30","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1038\/s41467-019-14198-8","volume":"11","author":"MY Batiuk","year":"2020","unstructured":"Batiuk MY, Martirosyan A, Wahis J, Vin F, Marneffe C, Kusserow C, Koeppen J, Viana JF, Oliveira JF, Voet T et al (2020) Identification of regionspecific astrocyte subtypes at single cell resolution. Nat Commun 11(1):1\u201315","journal-title":"Nat Commun"},{"issue":"1","key":"1871_CR31","doi-asserted-by":"publisher","first-page":"78","DOI":"10.1038\/s41593-018-0290-2","volume":"22","author":"C B\u00a8ottcher","year":"2019","unstructured":"B\u00a8ottcher C, Schlickeiser S, Sneeboer MA, Kunkel D, Knop A, Paza E, Fidzinski P, Kraus L, Snijders GJ, Kahn RS et al (2019) Human microglia regional heterogeneity and phenotypes determined by multiplexed single-cell mass cytometry. Nat Neurosci 22(1):78\u201390","journal-title":"Nat Neurosci"},{"issue":"7719","key":"1871_CR32","doi-asserted-by":"publisher","first-page":"494","DOI":"10.1038\/s41586-018-0414-6","volume":"560","author":"G La Manno","year":"2018","unstructured":"La Manno G, Soldatov R, Zeisel A, Braun E, Hochgerner H, Petukhov V, Lidschreiber K, Kastriti ME, L\u00f6nnerberg P, Furlan A, Fan J, Borm LE, Liu Z, Bruggen D, Guo J, He X, Barker R, Sundstr\u00f6m E, Castelo-Branco G, Cramer P, Adameyko I, Linnarsson S, Kharchenko PV (2018) RNA velocity of single cells. Nature 560(7719):494\u2013498. https:\/\/doi.org\/10.1038\/s41586-018-0414-6","journal-title":"Nature"},{"issue":"8","key":"1871_CR33","doi-asserted-by":"publisher","first-page":"1179","DOI":"10.1093\/bioinformatics\/btw777","volume":"33","author":"DJ McCarthy","year":"2017","unstructured":"McCarthy DJ, Campbell KR, Lun ATL, Wills QF (2017) Scater: Preprocessing, quality control, normalization and visualization of single-cell RNA-seq data in R. Bioinformatics 33(8):1179\u20131186. https:\/\/doi.org\/10.1093\/bioinformatics\/btw777","journal-title":"Bioinformatics"},{"key":"1871_CR34","doi-asserted-by":"publisher","first-page":"979","DOI":"10.12688\/f1000research.73600.2","volume":"10","author":"P-L Germain","year":"2022","unstructured":"Germain P-L, Lun A, Garcia Meixide C, Macnair W, Robinson MD (2022) Doublet identification in single-cell sequencing data using scDblFinder. F1000Research 10:979. https:\/\/doi.org\/10.12688\/f1000research.73600.2","journal-title":"F1000Research"},{"key":"1871_CR35","doi-asserted-by":"crossref","unstructured":"Lun ATL, Mccarthy DJ, Marioni JC (2016) A step-by-step workflow for low-level analysis of single-cell RNA-seq data with Bioconductor. F1000Research","DOI":"10.12688\/f1000research.9501.2"},{"issue":"13","key":"1871_CR36","doi-asserted-by":"publisher","first-page":"3573","DOI":"10.1016\/j.cell.2021.04.048","volume":"184","author":"Y Hao","year":"2021","unstructured":"Hao Y, Hao S, Andersen-Nissen E, Mauck WM, Zheng S, Butler A, Lee MJ, Wilk AJ, Darby C, Zager M, Hoffman P, Stoeckius M, Papalexi E, Mimitou EP, Jain J, Srivastava A, Stuart T, Fleming LM, Yeung B, Rogers AJ, McElrath JM, Blish CA, Gottardo R, Smibert P, Satija R (2021) Integrated analysis of multimodal single-cell data. Cell 184(13):3573\u2013358729. https:\/\/doi.org\/10.1016\/j.cell.2021.04.048","journal-title":"Cell"},{"key":"1871_CR37","doi-asserted-by":"publisher","unstructured":"Blondel VD, Guillaume JL, Lambiotte R, Lefebvre E (2008) Fast unfolding of communities in large networks. J Stat Mech: Theory Exp 2008(10). https:\/\/doi.org\/10.1088\/1742-5468\/2008\/10\/P10008 arXiv:0803.0476","DOI":"10.1088\/1742-5468\/2008\/10\/"},{"issue":"1","key":"1871_CR38","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/s13059-015-0844-5","volume":"16","author":"G Finak","year":"2015","unstructured":"Finak G, McDavid A, Yajima M, Deng J, Gersuk V, Shalek AK, Slichter CK, Miller HW, McElrath MJ, Prlic M, Linsley PS, Gottardo R (2015) MAST: a flexible statistical framework for assessing transcriptional changes and characterizing heterogeneity in single-cell RNA sequencing data. Genome Biol 16(1):1\u201313. https:\/\/doi.org\/10.1186\/s13059-015-0844-5","journal-title":"Genome Biol"},{"issue":"2","key":"1871_CR39","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1038\/s41587-021-01033-z","volume":"40","author":"E Dann","year":"2022","unstructured":"Dann E, Henderson NC, Teichmann SA, Morgan MD, Marioni JC (2022) Differential abundance testing on single-cell data using k-nearest neighbor graphs. Nat Biotechnol 40(2):245\u2013253","journal-title":"Nat Biotechnol"},{"issue":"1","key":"1871_CR40","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1038\/s41598-017-17204-5","volume":"7","author":"P Bankhead","year":"2017","unstructured":"Bankhead P, Loughrey MB, Fern\u00b4andez JA, Dombrowski Y, McArt DG, Dunne PD, McQuaid S, Gray RT, Murray LJ, Coleman HG, James JA, Salto-Tellez M, Hamilton PW (2017) QuPath: open source software for digital pathology image analysis. Sci Rep 7(1):1\u20137. https:\/\/doi.org\/10.1038\/s41598-017-17204-5","journal-title":"Sci Rep"},{"issue":"7745","key":"1871_CR41","doi-asserted-by":"publisher","first-page":"543","DOI":"10.1038\/s41586-019-0903-2","volume":"566","author":"S J\u00e4kel","year":"2019","unstructured":"J\u00e4kel S, Agirre E, Mendanha Falc\u00e3o A, Bruggen D, Lee KW, Knuesel I, Malhotra D, Ffrench-Constant C, Williams A, Castelo-Branco G (2019) Altered human oligodendrocyte heterogeneity in multiple sclerosis. Nature 566(7745):543\u2013547. https:\/\/doi.org\/10.1038\/s41586-019-0903-2","journal-title":"Nature"},{"issue":"7761","key":"1871_CR42","doi-asserted-by":"publisher","first-page":"332","DOI":"10.1038\/s41586-019-1195-2","volume":"570","author":"H Mathys","year":"2019","unstructured":"Mathys H, Davila-Velderrain J, Peng Z, Gao F, Mohammadi S, Young JZ, Menon M, He L, Abdurrob F, Jiang X, Martorell AJ, Ransohoff RM, Hafler BP, Bennett DA, Kellis M, Tsai LH (2019) Single-cell transcriptomic analysis of Alzheimer\u2019s disease. Nature 570(7761):332\u2013337. https:\/\/doi.org\/10.1038\/s41586-019-1195-2","journal-title":"Nature"},{"issue":"2","key":"1871_CR43","doi-asserted-by":"publisher","first-page":"316","DOI":"10.1016\/j.celrep.2017.09.050","volume":"21","author":"RB Tripathi","year":"2017","unstructured":"Tripathi RB, Jackiewicz M, McKenzie IA, Kougioumtzidou E, Grist M, Richardson WD (2017) Remarkable stability of myelinating oligodendrocytes in mice. Cell Rep 21(2):316\u2013323. https:\/\/doi.org\/10.1016\/j.celrep.2017.09.050","journal-title":"Cell Rep"},{"issue":"6291","key":"1871_CR44","doi-asserted-by":"publisher","first-page":"1326","DOI":"10.1126\/science.aaf6463","volume":"352","author":"S Marques","year":"2016","unstructured":"Marques S, Zeisel A, Codeluppi S, Van Bruggen D, Falcao AM, Xiao L, Li H, H\u00e4ring M, Hochgerner H, Romanov RA, Gyllborg D, Mu\u00f1oz-Manchado AB, La Manno G, L\u00f6nnerberg P, Floriddia EM, Rezayee F, Ernfors P, Arenas E, Hjerling-Leffler J, Harkany T, Richardson WD, Linnarsson S, Castelo-Branco G (2016) Oligodendrocyte heterogeneity in the mouse juvenile and adult central nervous system. Science 352(6291):1326\u20131329. https:\/\/doi.org\/10.1126\/science.aaf6463","journal-title":"Science"},{"issue":"9","key":"1871_CR45","doi-asserted-by":"publisher","first-page":"111291","DOI":"10.1016\/j.celrep.2022.111291","volume":"40","author":"A Benraiss","year":"2022","unstructured":"Benraiss A, Mariani JN, Tate A, Madsen PM, Clark KM, Welle KA, Solly R, Capellano L, Bentley K, Chandler-Militello D, Goldman SA (2022) A tcf7l2-responsive suppression of both homeostatic and compensatory remyelination in Huntington disease mice. Cell Rep 40(9):111291. https:\/\/doi.org\/10.1016\/j.celrep.2022.111291","journal-title":"Cell Rep"},{"issue":"7","key":"1871_CR46","doi-asserted-by":"publisher","first-page":"1276","DOI":"10.1016\/j.cell.2017.05.018","volume":"169","author":"H Keren-Shaul","year":"2017","unstructured":"Keren-Shaul H, Spinrad A, Weiner A, Matcovitch-Natan O, Dvir-Szternfeld R, Ulland TK, David E, Baruch K, Lara-Astaiso D, Toth B, Itzkovitz S, Colonna M, Schwartz M, Amit I (2017) A Unique Microglia Type Associated with Restricting Development of Alzheimer\u2019s Disease. Cell 169(7):1276\u2013129017. https:\/\/doi.org\/10.1016\/j.cell.2017.05.018","journal-title":"Cell"},{"issue":"3","key":"1871_CR47","doi-asserted-by":"publisher","first-page":"041371","DOI":"10.1101\/cshperspect.a041371","volume":"16","author":"RJ Franklin","year":"2024","unstructured":"Franklin RJ, Bodini B, Goldman SA (2024) Remyelination in the central nervous system. Cold Spring Harb Perspect Biol 16(3):041371","journal-title":"Cold Spring Harb Perspect Biol"},{"key":"1871_CR48","doi-asserted-by":"publisher","unstructured":"Macnair W, Calini D, Agirre E, Bryois J, J\u00e4kel S, Kukanja P, Stokar N, Ott V, Foo LC, Collin L, Schippling S, Urich E, Nutma E, Marzin M, Amor S, Magliozzi R, Heidari E, Robinson M, ffrench-Constant C, Castelo-Branco G, Williams A, Malhotra D (2023) Single nuclei rnaseq stratifies multiple sclerosis patients into distinct white matter glial responses. bioRxiv. https:\/\/doi.org\/10.1101\/2022.04.06.487263. https:\/\/www.biorxiv.org\/content\/early\/2023\/09\/20\/2022.04.06.487263.full.pdf","DOI":"10.1101\/2022.04.06.487263"},{"key":"1871_CR49","doi-asserted-by":"publisher","first-page":"3449","DOI":"10.1007\/s00018-013-1340-2","volume":"70","author":"E Medina-Rodr\u00edguez","year":"2013","unstructured":"Medina-Rodr\u00edguez E, Arenzana F, Pastor J, Redondo M, Palomo V, Garc\u00eda de Sola R, Gil C, Mart\u00ednez A, Bribi\u00e1n A, de Castro F (2013) Inhibition of endogenous phosphodiesterase 7 promotes oligodendrocyte precursor differentiation and survival. Cell Mol Life Sci 70:3449\u20133462","journal-title":"Cell Mol Life Sci"},{"issue":"12","key":"1871_CR50","doi-asserted-by":"publisher","first-page":"1918","DOI":"10.1002\/emmm.201303123","volume":"5","author":"YA Syed","year":"2013","unstructured":"Syed YA, Baer A, Hofer MP, Gonz\u00b4alez GA, Rundle J, Myrta S, Huang JK, Zhao C, Rossner MJ, Trotter MW et al (2013) Inhibition of phosphodiesterase-4 promotes oligodendrocyte precursor cell differentiation and enhances cns remyelination. EMBO Mol Med 5(12):1918\u20131934","journal-title":"EMBO Mol Med"},{"issue":"2","key":"1871_CR51","doi-asserted-by":"publisher","first-page":"299","DOI":"10.1038\/jcbfm.2009.210","volume":"30","author":"N Miyamoto","year":"2009","unstructured":"Miyamoto N, Tanaka R, Shimura H, Watanabe T (2009) Phosphodiesterase III inhibition promotes differentiation and survival of oligodendrocyte progenitors and enhances regeneration of ischemic white matter lesions in the adult mammalian brain. J Cereb Blood Flow Metabolism 30(2):299\u2013310. https:\/\/doi.org\/10.1038\/jcbfm.2009.210","journal-title":"J Cereb Blood Flow Metabolism"},{"issue":"10","key":"1871_CR52","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1371\/journal.pone.0013417","volume":"5","author":"C Giamp\u00e0","year":"2010","unstructured":"Giamp\u00e0 C, Laurenti D, Anzilotti S, Bernardi G, Menniti FS, Fusco FR (2010) Inhibition of the striatal specific phosphodiesterase pde10a ameliorates striatal and cortical pathology in r6\/2 mouse model of Huntington\u2019s disease. PLoS One 5(10):1\u201314. https:\/\/doi.org\/10.1371\/journal.pone.0013417","journal-title":"PLoS One"},{"key":"1871_CR53","doi-asserted-by":"publisher","first-page":"1220","DOI":"10.1016\/j.neuron.2016.10.064","volume":"92","author":"V Beaumont","year":"2016","unstructured":"Beaumont V, Zhong S, Lin H, Schmidt CJ, Zaleska MM, Munoz-sanjuan I, Beaumont V, Zhong S, Lin H, Xu W, Bradaia A, Steidl E, Gleyzes M (2016) Phosphodiesterase 10A inhibition improves cortico-basal ganglia function in Huntington\u2019s Disease models. Neuron 92:1220\u20131237. https:\/\/doi.org\/10.1016\/j.neuron.2016.10.064","journal-title":"Neuron"},{"issue":"Suppl 1","key":"1871_CR54","doi-asserted-by":"publisher","first-page":"99","DOI":"10.1136\/jnnp-2018-EHDN.266","volume":"89","author":"M Delnomdedieu","year":"2018","unstructured":"Delnomdedieu M, Tan Y, Ogden A, Berger Z, Reilmann R (2018) J06 a randomized, double-blind, placebo-controlled phase ii efficacy and safety study of the pde10a inhibitor pf-02545920 in Huntington disease (amaryllis). J Neurol Neurosurg Psychiatry 89(Suppl 1):99\u2013100. https:\/\/doi.org\/10.1136\/jnnp-2018-EHDN.266. https:\/\/jnnp.bmj.com\/content\/89\/Suppl1\/A99.3.full.pdf","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"2","key":"1871_CR55","doi-asserted-by":"publisher","first-page":"22","DOI":"10.3390\/jcdd5020022","volume":"5","author":"S Chen","year":"2018","unstructured":"Chen S, Knight WE, Yan C (2018) Roles of pde1 in pathological cardiac remodeling and dysfunction. J Cardiovasc Dev Disease 5(2):22","journal-title":"J Cardiovasc Dev Disease"},{"issue":"5","key":"1871_CR56","doi-asserted-by":"publisher","first-page":"5870","DOI":"10.1126\/sciadv.aaw5870","volume":"5","author":"H Zhang","year":"2019","unstructured":"Zhang H, Pan B, Wu P, Parajuli N, Rekhter MD, Goldberg AL, Wang X (2019) Pde1 inhibition facilitates proteasomal degradation of misfolded proteins and protects against cardiac proteinopathy. Sci Adv 5(5):5870","journal-title":"Sci Adv"},{"key":"1871_CR57","doi-asserted-by":"publisher","unstructured":"Lackie RE, Maciejewski A, Ostapchenko VG, Marques-Lopes J, Choy WY, Duennwald ML, Prado VF, Prado MAM (2017) The hsp70\/hsp90 chaperone machinery in neurodegenerative diseases. Front NeuroSci 11. https:\/\/doi.org\/10.3389\/fnins.2017.00254","DOI":"10.3389\/fnins.2017.00254"},{"issue":"29","key":"1871_CR58","doi-asserted-by":"publisher","first-page":"10261","DOI":"10.1073\/pnas.0503937102","volume":"102","author":"K Iijima-Ando","year":"2005","unstructured":"Iijima-Ando K, Wu P, Drier EA, Iijima K, Yin JCP (2005) cAMPresponse element-binding protein and heat-shock protein 70 additively suppress polyglutamine-mediated toxicity in Drosophila. Proc Natl Acad Sci USA 102(29):10261\u201310266. https:\/\/doi.org\/10.1073\/pnas.0503937102","journal-title":"Proc Natl Acad Sci USA"},{"issue":"1","key":"1871_CR59","doi-asserted-by":"publisher","first-page":"4692","DOI":"10.1038\/s41467-022-32370-5","volume":"13","author":"S Ayala Mariscal","year":"2022","unstructured":"Ayala Mariscal S, Pigazzini M, Richter Y, Ozel M, Grothaus I, Protze J, Ziege K, Kulke M, ElBediwi M, Vermaas J et al (2022) Identification of a httspecific binding motif in dnajb1 essential for suppression and disaggregation of htt. Nat Commun 13(1):4692","journal-title":"Nat Commun"},{"key":"1871_CR60","doi-asserted-by":"publisher","first-page":"108","DOI":"10.1016\/j.nbd.2018.10.017","volume":"124","author":"M Bason","year":"2019","unstructured":"Bason M, Meister-Broekema M, Alberts N, Dijkers P, Bergink S, Sibon OC, Kampinga HH (2019) Astrocytic expression of the chaperone dnajb6 results in non-cell autonomous protection in Huntington\u2019s disease. Neurobiol Dis 124:108\u2013117","journal-title":"Neurobiol Dis"},{"issue":"1","key":"1871_CR61","doi-asserted-by":"publisher","first-page":"7791","DOI":"10.1038\/s41467-022-35388-x","volume":"13","author":"RG Lim","year":"2022","unstructured":"Lim RG, Al-Dalahmah O, Wu J, Gold MP, Reidling JC, Tang G, Adam M, Dansu DK, Park H-J, Casaccia P et al (2022) Huntington disease oligodendrocyte maturation deficits revealed by single-nucleus rnaseq are rescued by thiamine-biotin supplementation. Nat Commun 13(1):7791","journal-title":"Nat Commun"},{"issue":"448","key":"1871_CR62","doi-asserted-by":"publisher","first-page":"9507","DOI":"10.1126\/scitranslmed.aam9507","volume":"10","author":"RM Rajani","year":"2018","unstructured":"Rajani RM, Quick S, Ruigrok SR, Graham D, Harris SE, Verhaaren BFJ, Fornage M, Seshadri S, Atanur SS, Dominiczak AF, Smith C, Wardlaw JM, Williams A (2018) Reversal of endothelial dysfunction reduces white matter vulnerability in cerebral small vessel disease in rats. Sci Transl Med 10(448):9507. https:\/\/doi.org\/10.1126\/scitranslmed.aam9507","journal-title":"Sci Transl Med"},{"issue":"3","key":"1871_CR63","doi-asserted-by":"publisher","first-page":"964","DOI":"10.1093\/brain\/awab446","volume":"145","author":"S Smaji\u0107","year":"2022","unstructured":"Smaji\u0107\u00a0S, Prada-Medina CA, Landoulsi Z, Ghelfi J, Delcambre S, Dietrich C, Jarazo J, Henck J, Balachandran S, Pachchek S, Morris CM, Antony P, Timmermann B, Sauer S, Pereira SL, Schwamborn JC, May P, Gru\u00a8newald A, Spielmann M (2022) Single-cell sequencing of human midbrain reveals glial activation and a Parkinson-specific neuronal state. Brain 145(3):964\u2013978. https:\/\/doi.org\/10.1093\/brain\/awab446","journal-title":"Brain"},{"issue":"5","key":"1871_CR64","doi-asserted-by":"publisher","first-page":"891","DOI":"10.1016\/j.neuron.2020.06.021","volume":"107","author":"H Lee","year":"2020","unstructured":"Lee H, Fenster RJ, Pineda SS, Gibbs WS, Mohammadi S, DavilaVelderrain J, Garcia FJ, Therrien M, Novis HS, Gao F, Wilkinson H, Vogt T, Kellis M, LaVoie MJ, Heiman M (2020) Cell type-specific Transcriptomics reveals that mutant huntingtin leads to mitochondrial RNA release and neuronal Innate Immune activation. Neuron 107(5):891\u20139088. https:\/\/doi.org\/10.1016\/j.neuron.2020.06.021","journal-title":"Neuron"},{"key":"1871_CR65","doi-asserted-by":"publisher","first-page":"1191324","DOI":"10.3389\/fnmol.2023.1191324","volume":"16","author":"J Gasser","year":"2023","unstructured":"Gasser J, Gillet G, Valadas JS, Rouvi`ere L, Kotian A, Fan W, Keaney J, Kadiu I (2023) Innate immune activation and aberrant function in the r6\/2 mouse model and Huntington\u2019s disease ipsc-derived microglia. Front Mol Neurosci 16:1191324","journal-title":"Front Mol Neurosci"},{"issue":"20","key":"1871_CR66","first-page":"8221","volume":"14","author":"P Dent","year":"2022","unstructured":"Dent P, Booth L, Roberts JL, Poklepovic A, Martinez J, Cridebring D, Reiman EM (2022) Ar12 increases bag3 expression which is essential for tau and app degradation via lc3-associated phagocytosis and macroautophagy. Aging 14(20):8221","journal-title":"Aging"},{"key":"1871_CR67","doi-asserted-by":"crossref","unstructured":"Ying Z-M, Lv Q-K, Yao X-Y, Dong A-Q, Yang Y-P, Cao Y-L, Wang F, Gong A-P, Liu C-F (2022) Bag3 promotes autophagy and suppresses nlrp3 inflammasome activation in parkinson\u2019s disease. Annals Translational Med 10(22)","DOI":"10.21037\/atm-22-5159"},{"issue":"1","key":"1871_CR68","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1038\/s41467-020-17876-0","volume":"11","author":"D Agarwal","year":"2020","unstructured":"Agarwal D, Sandor C, Volpato V, Caffrey TM, Monz\u00b4on-Sandoval J, Bowden R, Alegre-Abarrategui J, Wade-Martins R, Webber C (2020) A single-cell atlas of the human substantia nigra reveals cell-specific pathways associated with neurological disorders. Nat Commun 11(1):1\u201311. https:\/\/doi.org\/10.1038\/s41467-020-17876-0","journal-title":"Nat Commun"},{"issue":"6","key":"1871_CR69","doi-asserted-by":"publisher","first-page":"771","DOI":"10.1038\/s41593-020-0621-y","volume":"23","author":"C Nagy","year":"2020","unstructured":"Nagy C, Maitra M, Tanti A, Suderman M, Th\u00e9roux J-F, Davoli MA, Perlman K, Yerko V, Wang YC, Tripathy SJ, et al (2020) Single-nucleus transcriptomics of the prefrontal cortex in major depressive disorder implicates oligodendrocyte precursor cells and excitatory neurons. Nat Neurosci 23(6):771\u2013781","journal-title":"Nat Neurosci"},{"issue":"1","key":"1871_CR70","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1186\/s40478-020-0880-6","volume":"8","author":"O Al-Dalahmah","year":"2020","unstructured":"Al-Dalahmah O, Sosunov AA, Shaik A, Ofori K, Liu Y, Vonsattel JP, Adorjan I, Menon V, Goldman JE (2020) Single-nucleus RNA-seq identifies Huntington disease astrocyte states. Acta Neuropathol Commun 8(1):1\u201321. https:\/\/doi.org\/10.1186\/s40478-020-0880-6","journal-title":"Acta Neuropathol Commun"},{"key":"1871_CR71","doi-asserted-by":"publisher","unstructured":"Hartl FU (1995) Principles of chaperone-mediated protein folding. Philosophical transactions of the Royal Society of London. Ser B Biol Sci 348(1323):107\u2013112.  https:\/\/doi.org\/10.1098\/rstb.1995.0051","DOI":"10.1098\/rstb.1995.0051"}],"container-title":["Acta Neuropathologica Communications"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40478-024-01871-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s40478-024-01871-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40478-024-01871-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,10,21]],"date-time":"2024-10-21T14:06:59Z","timestamp":1729519619000},"score":1,"resource":{"primary":{"URL":"https:\/\/actaneurocomms.biomedcentral.com\/articles\/10.1186\/s40478-024-01871-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,10,21]]},"references-count":71,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["1871"],"URL":"https:\/\/doi.org\/10.1186\/s40478-024-01871-3","relation":{"has-preprint":[{"id-type":"doi","id":"10.1101\/2022.09.10.507291","asserted-by":"object"}]},"ISSN":["2051-5960"],"issn-type":[{"value":"2051-5960","type":"electronic"}],"subject":[],"published":{"date-parts":[[2024,10,21]]},"assertion":[{"value":"26 September 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"29 September 2024","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"21 October 2024","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Fresh frozen brain samples from HD and non-neurological control donors were provided by prospective donor schemes with full ethical approval for use in research and publication from the MRC Edinburgh Brain Bank (EBB; 16\/ES\/0084), the Netherlands Brain Bank (NBB; 2009\/148), and Leiden University Medical Center (LBB). Paraffin sections were provided by prospective donors schemes with full ethical approval for use in research and publication from the MRC Edinburgh Brain Bank (EBB; 16\/ES\/0084) and the London Neurodegenerative Diseases Brain Bank (LNDBB; 18\/WA\/0206).","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"165"}}