{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T13:24:03Z","timestamp":1790774643994,"version":"4.1.0"},"publisher-location":"New York, NY","reference-count":30,"publisher":"Springer New York","isbn-type":[{"value":"9781493997831","type":"print"},{"value":"9781493997848","type":"electronic"}],"license":[{"start":{"date-parts":[[2019,10,5]],"date-time":"2019-10-05T00:00:00Z","timestamp":1570233600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020]]},"DOI":"10.1007\/978-1-4939-9784-8_14","type":"book-chapter","created":{"date-parts":[[2019,10,4]],"date-time":"2019-10-04T10:03:23Z","timestamp":1570183403000},"page":"217-240","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Real Time Videomicroscopy and Semiautomated Analysis of Brain Cell Culture Models of Trinucleotide Repeat Expansion Diseases"],"prefix":"10.1007","author":[{"given":"Sandra O.","family":"Braz","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana M.","family":"Dinca","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Genevi\u00e8ve","family":"Gourdon","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M\u00e1rio","family":"Gomes-Pereira","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2019,10,5]]},"reference":[{"key":"14_CR1","doi-asserted-by":"publisher","first-page":"247","DOI":"10.1038\/nrg2748","volume":"11","author":"AR Spada La","year":"2010","unstructured":"La Spada AR, Taylor JP (2010) Repeat expansion disease: progress and puzzles in disease pathogenesis. Nat Rev Genet 11:247\u2013258","journal-title":"Nat Rev Genet"},{"key":"14_CR2","doi-asserted-by":"publisher","first-page":"3","DOI":"10.1007\/978-1-62703-411-1_1","volume":"1010","author":"H Budworth","year":"2013","unstructured":"Budworth H, McMurray CT (2013) A brief history of triplet repeat diseases. Methods Mol Biol 1010:3\u201317","journal-title":"Methods Mol Biol"},{"key":"14_CR3","doi-asserted-by":"publisher","first-page":"256","DOI":"10.1016\/j.gde.2012.02.002","volume":"22","author":"T Wang","year":"2012","unstructured":"Wang T, Bray SM, Warren ST (2012) New perspectives on the biology of fragile X syndrome. Curr Opin Genet Dev 22:256\u2013263","journal-title":"Curr Opin Genet Dev"},{"key":"14_CR4","doi-asserted-by":"publisher","first-page":"165","DOI":"10.1242\/dmm.008706","volume":"5","author":"A Martelli","year":"2012","unstructured":"Martelli A, Napierala M, Puccio H (2012) Understanding the genetic and molecular pathogenesis of Friedreich\u2019s ataxia through animal and cellular models. Dis Model Mech 5:165\u2013176","journal-title":"Dis Model Mech"},{"key":"14_CR5","doi-asserted-by":"publisher","first-page":"83","DOI":"10.1016\/S1474-4422(10)70245-3","volume":"10","author":"CA Ross","year":"2011","unstructured":"Ross CA, Tabrizi SJ (2011) Huntington\u2019s disease: from molecular pathogenesis to clinical treatment. Lancet Neurol 10:83\u201398","journal-title":"Lancet Neurol"},{"key":"14_CR6","doi-asserted-by":"publisher","first-page":"R116","DOI":"10.1093\/hmg\/ddr343","volume":"20","author":"G Sicot","year":"2011","unstructured":"Sicot G, Gourdon G, Gomes-Pereira M (2011) Myotonic dystrophy, when simple repeats reveal complex pathogenic entities: new findings and future challenges. Hum Mol Genet 20:R116\u2013R123","journal-title":"Hum Mol Genet"},{"key":"14_CR7","doi-asserted-by":"publisher","first-page":"R45","DOI":"10.1093\/hmg\/ddt371","volume":"22","author":"JD Cleary","year":"2013","unstructured":"Cleary JD, Ranum LP (2013) Repeat-associated non-ATG (RAN) translation in neurological disease. Hum Mol Genet 22:R45\u2013R51","journal-title":"Hum Mol Genet"},{"key":"14_CR8","doi-asserted-by":"publisher","first-page":"519","DOI":"10.3389\/fneur.2018.00519","volume":"9","author":"SO Braz","year":"2018","unstructured":"Braz SO, Acquaire J, Gourdon G, Gomes-Pereira M (2018) Of Mice and Men: Advances in the Understanding of Neuromuscular Aspects of Myotonic Dystrophy. Front Neurol 9:519","journal-title":"Front Neurol"},{"key":"14_CR9","doi-asserted-by":"publisher","first-page":"112","DOI":"10.5214\/ans.0972.7531.210308","volume":"21","author":"AA Rikani","year":"2014","unstructured":"Rikani AA, Choudhry Z, Choudhry AM, Rizvi N, Ikram H, Mobassarah NJ, Tulli S (2014) The mechanism of degeneration of striatal neuronal subtypes in Huntington disease. Ann Neurosci 21:112\u2013114","journal-title":"Ann Neurosci"},{"key":"14_CR10","doi-asserted-by":"publisher","first-page":"49","DOI":"10.1016\/j.neulet.2018.02.004","volume":"688","author":"M Huang","year":"2019","unstructured":"Huang M, Verbeek DS (2019) Why do so many genetic insults lead to Purkinje Cell degeneration and spinocerebellar ataxia? Neurosci Lett 688:49\u201357","journal-title":"Neurosci Lett"},{"key":"14_CR11","doi-asserted-by":"publisher","first-page":"785","DOI":"10.1016\/j.nbd.2005.05.017","volume":"20","author":"D Zala","year":"2005","unstructured":"Zala D, Benchoua A, Brouillet E, Perrin V, Gaillard MC, Zurn AD, Aebischer P, Deglon N (2005) Progressive and selective striatal degeneration in primary neuronal cultures using lentiviral vector coding for a mutant huntingtin fragment. Neurobiol Dis 20:785\u2013798","journal-title":"Neurobiol Dis"},{"key":"14_CR12","doi-asserted-by":"publisher","first-page":"11","DOI":"10.29245\/2572.942X\/2017\/7.1134","volume":"2","author":"MMK Wong","year":"2017","unstructured":"Wong MMK, Watson LM, Becker EBE (2017) Recent advances in modelling of cerebellar ataxia using induced pluripotent stem cells. J Neurol Neuromedicine 2:11\u201315","journal-title":"J Neurol Neuromedicine"},{"key":"14_CR13","doi-asserted-by":"publisher","first-page":"4508","DOI":"10.1523\/JNEUROSCI.5027-09.2010","volume":"30","author":"S Jacobs","year":"2010","unstructured":"Jacobs S, Doering LC (2010) Astrocytes prevent abnormal neuronal development in the fragile x mouse. J Neurosci 30:4508\u20134514","journal-title":"J Neurosci"},{"key":"14_CR14","doi-asserted-by":"publisher","first-page":"202","DOI":"10.1016\/j.neuroscience.2016.03.011","volume":"324","author":"S Jacobs","year":"2016","unstructured":"Jacobs S, Cheng C, Doering LC (2016) Hippocampal neuronal subtypes develop abnormal dendritic arbors in the presence of Fragile X astrocytes. Neuroscience 324:202\u2013217","journal-title":"Neuroscience"},{"key":"14_CR15","doi-asserted-by":"publisher","first-page":"100","DOI":"10.1016\/j.mcn.2017.02.008","volume":"80","author":"C Franco","year":"2017","unstructured":"Franco C, Genis L, Navarro JA, Perez-Domper P, Fernandez AM, Schneuwly S, Torres Aleman I (2017) A role for astrocytes in cerebellar deficits in frataxin deficiency: Protection by insulin-like growth factor I. Mol Cell Neurosci 80:100\u2013110","journal-title":"Mol Cell Neurosci"},{"key":"14_CR16","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/j.nbd.2014.12.017","volume":"76","author":"F Loria","year":"2015","unstructured":"Loria F, Diaz-Nido J (2015) Frataxin knockdown in human astrocytes triggers cell death and the release of factors that cause neuronal toxicity. Neurobiol Dis 76:1\u201312","journal-title":"Neurobiol Dis"},{"key":"14_CR17","doi-asserted-by":"publisher","first-page":"1302","DOI":"10.1038\/nn1750","volume":"9","author":"SK Custer","year":"2006","unstructured":"Custer SK, Garden GA, Gill N, Rueb U, Libby RT, Schultz C, Guyenet SJ, Deller T, Westrum LE, Sopher BL, La Spada AR (2006) Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport. Nat Neurosci 9:1302\u20131311","journal-title":"Nat Neurosci"},{"key":"14_CR18","doi-asserted-by":"publisher","first-page":"2718","DOI":"10.1016\/j.celrep.2017.06.006","volume":"19","author":"G Sicot","year":"2017","unstructured":"Sicot G, Servais L, Dinca DM, Leroy A, Prigogine C, Medja F, Braz SO, Huguet-Lachon A, Chhuon C, Nicole A, Gueriba N, Oliveira R, Dan B, Furling D, Swanson MS, Guerrera IC, Cheron G, Gourdon G, Gomes-Pereira M (2017) Downregulation of the Glial GLT1 Glutamate Transporter and Purkinje Cell Dysfunction in a Mouse Model of Myotonic Dystrophy. Cell Rep 19:2718\u20132729","journal-title":"Cell Rep"},{"key":"14_CR19","doi-asserted-by":"publisher","first-page":"494","DOI":"10.1021\/cn500100r","volume":"5","author":"BS Khakh","year":"2014","unstructured":"Khakh BS, Sofroniew MV (2014) Astrocytes and Huntington\u2019s disease. ACS Chem Nerosci 5:494\u2013496","journal-title":"ACS Chem Nerosci"},{"key":"14_CR20","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1016\/j.brainres.2009.12.077","volume":"1318","author":"HJ Wenzel","year":"2010","unstructured":"Wenzel HJ, Hunsaker MR, Greco CM, Willemsen R, Berman RF (2010) Ubiquitin-positive intranuclear inclusions in neuronal and glial cells in a mouse model of the fragile X premutation. Brain Res 1318:155\u2013166","journal-title":"Brain Res"},{"key":"14_CR21","doi-asserted-by":"publisher","first-page":"289","DOI":"10.1016\/j.neuroscience.2015.01.003","volume":"289","author":"M Cvetanovic","year":"2015","unstructured":"Cvetanovic M, Ingram M, Orr H, Opal P (2015) Early activation of microglia and astrocytes in mouse models of spinocerebellar ataxia type 1. Neuroscience 289:289\u2013299","journal-title":"Neuroscience"},{"key":"14_CR22","doi-asserted-by":"publisher","first-page":"2508","DOI":"10.1093\/hmg\/ddv016","volume":"24","author":"J Jin","year":"2015","unstructured":"Jin J, Peng Q, Hou Z, Jiang M, Wang X, Langseth AJ, Tao M, Barker PB, Mori S, Bergles DE, Ross CA, Detloff PJ, Zhang J, Duan W (2015) Early white matter abnormalities, progressive brain pathology and motor deficits in a novel knock-in mouse model of Huntington\u2019s disease. Hum Mol Genet 24:2508\u20132527","journal-title":"Hum Mol Genet"},{"key":"14_CR23","doi-asserted-by":"publisher","first-page":"3920","DOI":"10.1093\/hmg\/ddt246","volume":"22","author":"LK Pacey","year":"2013","unstructured":"Pacey LK, Xuan IC, Guan S, Sussman D, Henkelman RM, Chen Y, Thomsen C, Hampson DR (2013) Delayed myelination in a mouse model of fragile X syndrome. Hum Mol Genet 22:3920\u20133930","journal-title":"Hum Mol Genet"},{"key":"14_CR24","doi-asserted-by":"publisher","first-page":"148","DOI":"10.1016\/j.brainresbull.2006.10.029","volume":"72","author":"YF Tai","year":"2007","unstructured":"Tai YF, Pavese N, Gerhard A, Tabrizi SJ, Barker RA, Brooks DJ, Piccini P (2007) Imaging microglial activation in Huntington\u2019s disease. Brain Res Bull 72:148\u2013151","journal-title":"Brain Res Bull"},{"key":"14_CR25","doi-asserted-by":"publisher","first-page":"438","DOI":"10.1016\/j.nbd.2011.09.003","volume":"45","author":"S Franciosi","year":"2012","unstructured":"Franciosi S, Ryu JK, Shim Y, Hill A, Connolly C, Hayden MR, McLarnon JG, Leavitt BR (2012) Age-dependent neurovascular abnormalities and altered microglial morphology in the YAC128 mouse model of Huntington disease. Neurobiol Dis 45:438\u2013449","journal-title":"Neurobiol Dis"},{"key":"14_CR26","doi-asserted-by":"publisher","first-page":"4737","DOI":"10.1172\/JCI64484","volume":"122","author":"W Kwan","year":"2012","unstructured":"Kwan W, Trager U, Davalos D, Chou A, Bouchard J, Andre R, Miller A, Weiss A, Giorgini F, Cheah C, Moller T, Stella N, Akassoglou K, Tabrizi SJ, Muchowski PJ (2012) Mutant huntingtin impairs immune cell migration in Huntington disease. J Clin Invest 122:4737\u20134747","journal-title":"J Clin Invest"},{"key":"14_CR27","doi-asserted-by":"publisher","first-page":"676","DOI":"10.1038\/nmeth.2019","volume":"9","author":"J Schindelin","year":"2012","unstructured":"Schindelin J, Arganda-Carreras I, Frise E, Kaynig V, Longair M, Pietzsch T, Preibisch S, Rueden C, Saalfeld S, Schmid B, Tinevez JY, White DJ, Hartenstein V, Eliceiri K, Tomancak P, Cardona A (2012) Fiji: an open-source platform for biological-image analysis. Nat Methods 9:676\u2013682","journal-title":"Nat Methods"},{"key":"14_CR28","doi-asserted-by":"publisher","first-page":"1931","DOI":"10.1038\/nprot.2014.131","volume":"9","author":"R Gorelik","year":"2014","unstructured":"Gorelik R, Gautreau A (2014) Quantitative and unbiased analysis of directional persistence in cell migration. Nat Protoc 9:1931\u20131943","journal-title":"Nat Protoc"},{"key":"14_CR29","doi-asserted-by":"publisher","first-page":"219","DOI":"10.1016\/j.jneumeth.2012.06.017","volume":"209","author":"TA Dincman","year":"2012","unstructured":"Dincman TA, Beare JE, Ohri SS, Whittemore SR (2012) Isolation of cortical mouse oligodendrocyte precursor cells. J Neurosci Methods 209:219\u2013226","journal-title":"J Neurosci Methods"},{"key":"14_CR30","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1007\/978-1-4939-7701-7_2","volume":"1749","author":"N Molinie","year":"2018","unstructured":"Molinie N, Gautreau A (2018) Directional Collective Migration in Wound Healing Assays. Methods Mol Biol 1749:11\u201319","journal-title":"Methods Mol Biol"}],"container-title":["Methods in Molecular Biology","Trinucleotide Repeats"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/978-1-4939-9784-8_14","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2019,10,4]],"date-time":"2019-10-04T10:07:28Z","timestamp":1570183648000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/978-1-4939-9784-8_14"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,10,5]]},"ISBN":["9781493997831","9781493997848"],"references-count":30,"URL":"https:\/\/doi.org\/10.1007\/978-1-4939-9784-8_14","relation":{},"ISSN":["1064-3745","1940-6029"],"issn-type":[{"value":"1064-3745","type":"print"},{"value":"1940-6029","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,10,5]]},"assertion":[{"value":"5 October 2019","order":1,"name":"first_online","label":"First Online","group":{"name":"ChapterHistory","label":"Chapter History"}}]}}