{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,10]],"date-time":"2026-07-10T22:03:55Z","timestamp":1783721035400,"version":"3.55.0"},"reference-count":73,"publisher":"MDPI AG","issue":"20","license":[{"start":{"date-parts":[[2022,10,21]],"date-time":"2022-10-21T00:00:00Z","timestamp":1666310400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institute of Health NINDS","doi-asserted-by":"publisher","award":["R01 NS197387"],"award-info":[{"award-number":["R01 NS197387"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institute of Health NINDS","doi-asserted-by":"publisher","award":["R01 NS109077"],"award-info":[{"award-number":["R01 NS109077"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>While astrocyte heterogeneity is an important feature of the healthy brain, less is understood about spatiotemporal heterogeneity of astrocytes in brain disease. Spinocerebellar ataxia type 1 (SCA1) is a progressive neurodegenerative disease caused by a CAG repeat expansion in the gene Ataxin1 (ATXN1). We characterized astrocytes across disease progression in the four clinically relevant brain regions, cerebellum, brainstem, hippocampus, and motor cortex, of Atxn1154Q\/2Q mice, a knock-in mouse model of SCA1. We found brain region-specific changes in astrocyte density and GFAP expression and area, early in the disease and prior to neuronal loss. Expression of astrocytic core homeostatic genes was also altered in a brain region-specific manner and correlated with neuronal activity, indicating that astrocytes may compensate or exacerbate neuronal dysfunction. Late in disease, expression of astrocytic homeostatic genes was reduced in all four brain regions, indicating loss of astrocyte functions. We observed no obvious correlation between spatiotemporal changes in microglia and spatiotemporal astrocyte alterations, indicating a complex orchestration of glial phenotypes in disease. These results support spatiotemporal diversity of glial phenotypes as an important feature of the brain disease that may contribute to SCA1 pathogenesis in a brain region and disease stage-specific manner.<\/jats:p>","DOI":"10.3390\/cells11203323","type":"journal-article","created":{"date-parts":[[2022,10,23]],"date-time":"2022-10-23T20:43:50Z","timestamp":1666557830000},"page":"3323","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Spatial and Temporal Diversity of Astrocyte Phenotypes in Spinocerebellar Ataxia Type 1 Mice"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7452-8313","authenticated-orcid":false,"given":"Juao-Guilherme","family":"Rosa","sequence":"first","affiliation":[{"name":"Cvetanovic Laboratory, Department of Neuroscience, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"},{"name":"Graduate Program for Neuroscience, Boston University, 700 Albany Street W516, Boston, MA 02118, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katherine","family":"Hamel","sequence":"additional","affiliation":[{"name":"Cvetanovic Laboratory, Department of Neuroscience, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carrie","family":"Sheeler","sequence":"additional","affiliation":[{"name":"Cvetanovic Laboratory, Department of Neuroscience, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ella","family":"Borgenheimer","sequence":"additional","affiliation":[{"name":"Cvetanovic Laboratory, Department of Neuroscience, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"},{"name":"Baylor College of Medicine, 1 Baylor Plaza, Houston, TX 77030, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8952-6417","authenticated-orcid":false,"given":"Stephen","family":"Gilliat","sequence":"additional","affiliation":[{"name":"Cvetanovic Laboratory, Department of Neuroscience, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1164-973X","authenticated-orcid":false,"given":"Alyssa","family":"Soles","sequence":"additional","affiliation":[{"name":"Cvetanovic Laboratory, Department of Neuroscience, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ferris J.","family":"Ghannoum","sequence":"additional","affiliation":[{"name":"Cvetanovic Laboratory, Department of Neuroscience, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kaelin","family":"Sbrocco","sequence":"additional","affiliation":[{"name":"Cvetanovic Laboratory, Department of Neuroscience, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5757-4548","authenticated-orcid":false,"given":"Hillary P.","family":"Handler","sequence":"additional","affiliation":[{"name":"Cvetanovic Laboratory, Department of Neuroscience, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"},{"name":"Institute for Translational Neuroscience, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Orion","family":"Rainwater","sequence":"additional","affiliation":[{"name":"Institute for Translational Neuroscience, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"},{"name":"Department of Lab. Medicine and Pathology, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7573-5453","authenticated-orcid":false,"given":"Ryan","family":"Kang","sequence":"additional","affiliation":[{"name":"Cvetanovic Laboratory, Department of Neuroscience, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"},{"name":"Bowdoin College, Brunswick, ME 04011, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marija","family":"Cvetanovic","sequence":"additional","affiliation":[{"name":"Cvetanovic Laboratory, Department of Neuroscience, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"},{"name":"Institute for Translational Neuroscience, College of Medicine, University of Minnesota, 2101 6th Street SE, Minneapolis, MN 55455, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,10,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Sheeler, C., Rosa, J., Ferro, A., Mcadams, B., Borgenheimer, E., and Cvetanovic, M. (2020). Glia in Neurodegeneration: The Housekeeper, the Defender and the Perpetrator. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21239188"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"430","DOI":"10.1016\/j.neuron.2008.10.013","article-title":"The Mystery and Magic of Glia: A Perspective on Their Roles in Health and Disease","volume":"60","author":"Barres","year":"2008","journal-title":"Neuron"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1016\/j.tins.2015.07.006","article-title":"Do stars govern our actions? Astrocyte involvement in rodent behavior","volume":"38","author":"Oliveira","year":"2015","journal-title":"Trends Neurosci."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1002\/glia.22299","article-title":"Relationship between glial potassium regulation and axon excitability: A role for glial Kir4.1 channels","volume":"60","author":"Bay","year":"2012","journal-title":"Glia"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"274","DOI":"10.1002\/glia.20455","article-title":"Downregulation of Kir4.1 inward rectifying potassium channel subunits by RNAi impairs potassium transfer and glutamate uptake by cultured cortical as-trocytes","volume":"55","author":"Kucheryavykh","year":"2007","journal-title":"Glia"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"7438","DOI":"10.1073\/pnas.1617330114","article-title":"Glutamate transporter GLAST controls synaptic wrapping by Bergmann glia and ensures proper wiring of Purkinje cells","volume":"114","author":"Miyazaki","year":"2017","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"1543","DOI":"10.1152\/physrev.00011.2013","article-title":"Physiological Roles of Aquaporin-4 in Brain","volume":"93","author":"Nagelhus","year":"2013","journal-title":"Physiol. Rev."},{"key":"ref_8","first-page":"161","article-title":"Heterogeneity of heterogeneities in neuronal networks. Front. Comput","volume":"8","author":"Baroni","year":"2014","journal-title":"Neurosci."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"eabe8978","DOI":"10.1126\/sciadv.abe8978","article-title":"Astrocytes and neurons share region-specific transcriptional signatures that confer regional identity to neuronal reprogramming","volume":"7","author":"Espinosa","year":"2021","journal-title":"Sci. Adv."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1126\/science.aaa7945","article-title":"Glial Cell Signaling. Circuit-specific signaling in astro-cyte-neuron networks in basal ganglia pathways","volume":"349","author":"Moratalla","year":"2015","journal-title":"Science"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"1138","DOI":"10.1126\/science.aaa1934","article-title":"Cell types in the mouse cortex and hippocampus revealed by single-cell RNA-seq","volume":"347","author":"Zeisel","year":"2015","journal-title":"Science"},{"key":"ref_12","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":"ref_13","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."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"eabf1230","DOI":"10.1126\/science.abf1230","article-title":"Barcoded viral tracing of single-cell interactions in central nervous system inflammation","volume":"372","author":"Clark","year":"2021","journal-title":"Science"},{"key":"ref_15","doi-asserted-by":"crossref","unstructured":"Pestana, F., Edwards-Faret, G., Belgard, T.G., Martirosyan, A., and Holt, M.G. (2020). No Longer Underappreciated: The Emerging Concept of Astrocyte Heterogeneity in Neuroscience. Brain Sci., 10.","DOI":"10.3390\/brainsci10030168"},{"key":"ref_16","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."},{"key":"ref_17","first-page":"31","article-title":"Functional diversity of astrocytes in neural circuit regulation","volume":"18","author":"Rowitch","year":"2016","journal-title":"Nat. Rev. Neurosci."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1016\/j.neulet.2013.12.071","article-title":"The dual role of astrocyte activation and reactive gliosis","volume":"565","author":"Pekny","year":"2014","journal-title":"Neurosci. Lett."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1038\/s41573-022-00390-x","article-title":"Function and therapeutic value of astrocytes in neurological diseases","volume":"21","author":"Lee","year":"2022","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"eaaw8546","DOI":"10.1126\/scitranslmed.aaw8546","article-title":"Astrocyte molecular signatures in Huntington\u2019s disease","volume":"11","author":"Gangwani","year":"2019","journal-title":"Sci. Transl. Med."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1038\/nn.3691","article-title":"Astrocyte Kir4.1 ion channel deficits contribute to neuronal dys-function in Huntington\u2019s disease model mice","volume":"17","author":"Tong","year":"2014","journal-title":"Nat. Neurosci."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"701","DOI":"10.1038\/s41593-020-0624-8","article-title":"Disease-associated astrocytes in Alzheimer\u2019s disease and aging","volume":"23","author":"Habib","year":"2020","journal-title":"Nat. Neurosci."},{"key":"ref_23","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":"ref_24","doi-asserted-by":"crossref","first-page":"59","DOI":"10.3389\/fnagi.2019.00059","article-title":"Astrocyte Heterogeneity: Impact to Brain Aging and Disease","volume":"11","author":"Matias","year":"2019","journal-title":"Front. Aging Neurosci."},{"key":"ref_25","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":"ref_26","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/j.neuron.2011.06.031","article-title":"Selective Neuronal Vulnerability in Neurodegenerative Diseases: From Stressor Thresholds to Degeneration","volume":"71","author":"Saxena","year":"2011","journal-title":"Neuron"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"838","DOI":"10.4103\/1673-5374.268897","article-title":"Regional brain susceptibility to neurodegeneration: What is the role of glial cells?","volume":"15","author":"Cragnolini","year":"2020","journal-title":"Neural Regen. Res."},{"key":"ref_28","first-page":"221","article-title":"Expansion of an unstable trinucleotide CAG repeat in spinocerebellar ataxia type Nat","volume":"4","author":"Orr","year":"1993","journal-title":"Genet."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"7425","DOI":"10.1074\/jbc.R800041200","article-title":"Pathogenic Mechanisms of a Polyglutamine-mediated Neurodegenerative Disease, Spinocerebellar Ataxia Type 1","volume":"284","author":"Zoghbi","year":"2009","journal-title":"J. Biol. Chem."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1007\/s12311-010-0183-8","article-title":"Neuropsychological features of patients with spinocerebellar ataxia (SCA) types 1, 2, 3, and 6","volume":"9","author":"Klinke","year":"2010","journal-title":"Cerebellum"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"580","DOI":"10.1002\/ana.410230609","article-title":"Spinocerebellar ataxia: Variable age of onset and linkage to human leukocyte antigen in a large kindred","volume":"23","author":"Zoghbi","year":"1988","journal-title":"Ann. Neurol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1080\/14734220510007950","article-title":"The pathogenesis of spinocerebellar ataxia","volume":"4","author":"Koeppen","year":"2005","journal-title":"Cerebellum"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"e123193","DOI":"10.1172\/jci.insight.123193","article-title":"Antisense oligonucleotide\u2013mediated ataxin-1 reduction prolongs survival in SCA1 mice and reveals disease-associated transcriptome profiles","volume":"3","author":"Friedrich","year":"2018","journal-title":"JCI Insight"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"665","DOI":"10.1111\/j.1365-2990.2012.01259.x","article-title":"Spinocerebellar ataxia type 1 (SCA1): New pathoana-tomical and clinico-pathological insights","volume":"38","author":"Timmann","year":"2012","journal-title":"Neuropathol. Appl. Neurobiol."},{"key":"ref_35","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."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"1220","DOI":"10.1002\/mds.27739","article-title":"Prediction of Survival With Long-Term Disease Progression in Most Common Spinocerebellar Ataxia","volume":"34","author":"Diallo","year":"2019","journal-title":"Mov. Disord."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1186\/s13023-016-0447-6","article-title":"A longitudinal investigation into cognition and disease pro-gression in spinocerebellar ataxia types 1, 2, 3, 6, and 7","volume":"11","author":"Moriarty","year":"2016","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"650","DOI":"10.1016\/S1474-4422(13)70104-2","article-title":"Biological and clinical characteristics of individuals at risk for spinocerebellar ataxia types 1, 2, 3, and 6 in the longitudinal RISCA study: Analysis of baseline data","volume":"12","author":"Jacobi","year":"2013","journal-title":"Lancet Neurol."},{"key":"ref_39","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."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1212\/WNL.45.1.24","article-title":"Clinical, neuropathologic, and genetic studies of a large spinocerebellar ataxia type 1 (SCA1) kindred: (CAG)n expansion and early premonitory signs and symptoms","volume":"45","author":"Genis","year":"1995","journal-title":"Neurology"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1016\/j.pneurobio.2013.01.001","article-title":"Clinical features, neurogenetics and neu-ropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7","volume":"104","author":"Paulson","year":"2013","journal-title":"Prog. Neurobiol."},{"key":"ref_42","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"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1093\/hmg\/ddz265","article-title":"Cerebellar contribution to the cognitive alterations in SCA1: Evidence from mouse models","volume":"29","author":"Asher","year":"2019","journal-title":"Hum. Mol. Genet."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1159","DOI":"10.1016\/j.cell.2019.07.043","article-title":"Loss of Ataxin-1 Potentiates Alzheimer\u2019s Pathogenesis by Elevating Cerebral BACE1 Transcription","volume":"178","author":"Suh","year":"2019","journal-title":"Cell"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1007\/s12311-016-0794-9","article-title":"Mutant Ataxin-1 Inhibits Neural Progenitor Cell Proliferation in SCA1","volume":"16","author":"Cvetanovic","year":"2016","journal-title":"Cerebellum"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1445","DOI":"10.1038\/nm.2494","article-title":"Vascular endothelial growth factor ameliorates the ataxic phenotype in a mouse model of spinocerebellar ataxia type 1","volume":"17","author":"Cvetanovic","year":"2011","journal-title":"Nat. Med."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"8853","DOI":"10.1523\/JNEUROSCI.2978-04.2004","article-title":"Recovery from polyglutamine-induced neurodegen-eration in conditional SCA1 transgenic mice","volume":"24","author":"Zu","year":"2004","journal-title":"J. Neurosci."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s00401-016-1553-1","article-title":"The role of glial-specific Kir4.1 in normal and pathological states of the CNS","volume":"132","author":"Nwaobi","year":"2016","journal-title":"Acta Neuropathol."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1016\/S0896-6273(00)80086-0","article-title":"Antisense knockout of glutamate transporters reveals a predominant role for astroglial glutamate transport in excitotoxicity and clearance of extracellular glutamate","volume":"16","author":"Rothstein","year":"1996","journal-title":"Neuron"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"1365","DOI":"10.1016\/j.neuron.2017.08.022","article-title":"Connexin 43-Mediated Astroglial Metabolic Networks Contribute to the Regulation of the Sleep-Connexin 43-Mediated Astroglial Metabolic Networks Contribute to the Regulation of the Sleep-Wake Cycle","volume":"95","author":"Clasadonte","year":"2017","journal-title":"Neuron"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1038\/nrg1691","article-title":"Diseases of unstable repeat expansion: Mechanisms and common principles","volume":"6","author":"Gatchel","year":"2005","journal-title":"Nat. Rev. Genet."},{"key":"ref_52","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":"ref_53","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1093\/hmg\/5.1.33","article-title":"Cloning and developmental expression analysis of the murine homolog of the spinocerebellar ataxia type 1 gene (Sca1)","volume":"5","author":"Matilla","year":"1996","journal-title":"Hum. Mol. Genet."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1016\/j.neuroscience.2016.02.011","article-title":"Ataxin-1 regulates proliferation of hippocampal neural precursors","volume":"322","author":"Asher","year":"2016","journal-title":"Neuroscience"},{"key":"ref_55","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."},{"key":"ref_56","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."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1007\/s00401-015-1513-1","article-title":"Astrocytes: A central element in neurological diseases","volume":"131","author":"Pekny","year":"2015","journal-title":"Acta Neuropathol."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1017\/S1740925X10000165","article-title":"Bergmann glial ensheathment of dendritic spines regulates synapse number without affecting spine motility","volume":"6","author":"Lordkipanidze","year":"2010","journal-title":"Neuron Glia Biol."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"849","DOI":"10.1126\/science.aab3103","article-title":"Neurons diversify astrocytes in the adult brain through sonic hedgehog signaling","volume":"351","author":"Farmer","year":"2016","journal-title":"Science"},{"key":"ref_60","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"},{"key":"ref_61","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."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"378","DOI":"10.3389\/fncel.2014.00378","article-title":"Neuron-glia networks: Integral gear of brain function","volume":"8","author":"Perea","year":"2014","journal-title":"Front. Cell Neurosci."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"1045","DOI":"10.1016\/j.neuroscience.2004.06.008","article-title":"Potassium buffering in the central nervous system","volume":"129","author":"Kofuji","year":"2004","journal-title":"Neuroscience"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"11354","DOI":"10.1523\/JNEUROSCI.0723-07.2007","article-title":"Conditional Knock-Out of Kir4.1 Leads to Glial Membrane Depolarization, Inhibition of Potassium and Glutamate Uptake, and Enhanced Short-Term Synaptic Potentiation","volume":"27","author":"Djukic","year":"2007","journal-title":"J. Neurosci."},{"key":"ref_65","doi-asserted-by":"crossref","unstructured":"Ferro, A., Qu, W., Lukowicz, A., Svedberg, D., Johnson, A., and Cvetanovic, M. (2018). Inhibition of NF-\u03baB signaling in IKK\u03b2 F\/F.;LysM Cre mice causes motor deficits but does not alter pathogenesis of Spinocerebellar ataxia type 1. PLoS ONE, 1.","DOI":"10.1371\/journal.pone.0200013"},{"key":"ref_66","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. Neuroinflamm."},{"key":"ref_67","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"},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1038\/nature25752","article-title":"Astroglial Kir4.1 in the lateral habenula drives neuronal bursts in depression","volume":"554","author":"Cui","year":"2018","journal-title":"Nature"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"306","DOI":"10.1016\/j.neuron.2018.03.010","article-title":"Kir4.1-Dependent Astrocyte-Fast Motor Neuron Interactions Are Required for Peak Strength","volume":"98","author":"Kelley","year":"2018","journal-title":"Neuron"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"611494","DOI":"10.3389\/fnagi.2020.611494","article-title":"The Involvement of Aquaporin-4 in the Interstitial Fluid Drainage Impairment Following Subarachnoid Hemorrhage","volume":"12","author":"Liu","year":"2021","journal-title":"Front. Aging Neurosci."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"212","DOI":"10.1002\/glia.20275","article-title":"Glutamate-mediated glial injury: Mechanisms and clinical importance","volume":"53","author":"Matute","year":"2005","journal-title":"Glia"},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1016\/j.expneurol.2006.03.028","article-title":"Loss of the astrocyte glutamate transporter GLT1 modifies disease in SOD1G93A mice","volume":"201","author":"Pardo","year":"2006","journal-title":"Exp. Neurol."},{"key":"ref_73","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."}],"container-title":["Cells"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2073-4409\/11\/20\/3323\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T00:59:05Z","timestamp":1760144345000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2073-4409\/11\/20\/3323"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,10,21]]},"references-count":73,"journal-issue":{"issue":"20","published-online":{"date-parts":[[2022,10]]}},"alternative-id":["cells11203323"],"URL":"https:\/\/doi.org\/10.3390\/cells11203323","relation":{"has-preprint":[{"id-type":"doi","id":"10.1101\/2021.09.13.460129","asserted-by":"object"}]},"ISSN":["2073-4409"],"issn-type":[{"value":"2073-4409","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,10,21]]}}}