{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,13]],"date-time":"2026-11-13T02:37:26Z","timestamp":1794537446118,"version":"4.9.1"},"reference-count":47,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2019,12,1]],"date-time":"2019-12-01T00:00:00Z","timestamp":1575158400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2019,12,30]],"date-time":"2019-12-30T00:00:00Z","timestamp":1577664000000},"content-version":"vor","delay-in-days":29,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"the KBRI Basic Research Program funded by the Ministry of Science and ICT of Republic of Korea","award":["19-BR-01-02"],"award-info":[{"award-number":["19-BR-01-02"]}]},{"name":"the NRF Brain Research Program funded by the Ministry of Science and ICT of Republic of Korea","award":["2017M3C7A 1048086"],"award-info":[{"award-number":["2017M3C7A 1048086"]}]},{"name":"the NRF Brain Research Program funded by the Ministry of Science and ICT of Republic of Korea","award":["2017M3C7A1027472"],"award-info":[{"award-number":["2017M3C7A1027472"]}]},{"DOI":"10.13039\/501100003625","name":"Ministry of Health and Welfare","doi-asserted-by":"publisher","award":["H18C1254"],"award-info":[{"award-number":["H18C1254"]}],"id":[{"id":"10.13039\/501100003625","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Mol Brain"],"published-print":{"date-parts":[[2019,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec>\n                <jats:title>Background<\/jats:title>\n                <jats:p>Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare form of infantile-onset leukodystrophy. The disorder is caused primarily by mutations of <jats:italic>MLC1<\/jats:italic> that leads to a series of phenotypic outcomes including vacuolation of myelin and astrocytes, subcortical cysts, brain edema, and macrocephaly. Recent studies have indicated that functional interactions among MLC1, GlialCAM, and ClC-2 channels play key roles in the regulation of neuronal, glial and vascular homeostasis. However, the physiological role of MLC1 in cellular homeostatic communication remains poorly understood. In the present study, we investigated the cellular function of MLC1 and its effects on cell\u2013cell interactions.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>MLC1-dependent cellular morphology and motility were analyzed by using confocal and live cell imaging technique. Biochemical approaches such as immunoblotting, co-immunoprecipitation, and surface biotinylation were conducted to support data.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>We found that the altered MLC1 expression and localization led to a great alteration in cellular morphology and motility through actin remodeling. MLC1 overexpression induced filopodia formation and suppressed motility. And, MLC1 proteins expressed in patient-derived <jats:italic>MLC1<\/jats:italic> mutants resulted in trapping in the ER although no changes in morphology or motility were observed. Interestingly knockdown of <jats:italic>Mlc1<\/jats:italic> induced Arp3-Cortactin interaction, lamellipodia formation, and increased the membrane ruffling of the astrocytes. These data indicate that subcellular localization of expressed MLC1 at the plasma membrane is critical for changes in actin dynamics through ARP2\/3 complex. Thus, our results suggest that misallocation of pathogenic mutant MLC1 may disturbs the stable cell-cell communication and the homeostatic regulation of astrocytes in patients with MLC.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1186\/s13041-019-0540-6","type":"journal-article","created":{"date-parts":[[2019,12,30]],"date-time":"2019-12-30T16:02:51Z","timestamp":1577721771000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Plasma membrane localization of MLC1 regulates cellular morphology and motility"],"prefix":"10.1186","volume":"12","author":[{"given":"Junmo","family":"Hwang","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hung M.","family":"Vu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min-Sik","family":"Kim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5477-5640","authenticated-orcid":false,"given":"Hyun-Ho","family":"Lim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2019,12,30]]},"reference":[{"key":"540_CR1","doi-asserted-by":"publisher","first-page":"679","DOI":"10.1007\/BF00593394","volume":"37","author":"MS van der Knaap","year":"1995","unstructured":"van der Knaap MS, Valk J, Barth PG, Smit LM, van Engelen BG, Tortori Donati P. Leukoencephalopathy with swelling in children and adolescents: MRI patterns and differential diagnosis. Neuroradiology. 1995;37:679\u201386.","journal-title":"Neuroradiology"},{"key":"540_CR2","doi-asserted-by":"publisher","first-page":"973","DOI":"10.1016\/S1474-4422(12)70192-8","volume":"11","author":"MS van der Knaap","year":"2012","unstructured":"van der Knaap MS, Boor I, Estevez R. Megalencephalic leukoencephalopathy with subcortical cysts: chronic white matter oedema due to a defect in brain ion and water homoeostasis. Lancet Neurol. 2012;11:973\u201385.","journal-title":"Lancet Neurol"},{"key":"540_CR3","doi-asserted-by":"publisher","first-page":"324","DOI":"10.1002\/ana.410370308","volume":"37","author":"MS van der Knaap","year":"1995","unstructured":"van der Knaap MS, Barth PG, Stroink H, van Nieuwenhuizen O, Arts WF, Hoogenraad F, Valk J. Leukoencephalopathy with swelling and a discrepantly mild clinical course in eight children. Ann Neurol. 1995;37:324\u201334.","journal-title":"Ann Neurol"},{"key":"540_CR4","doi-asserted-by":"publisher","first-page":"114","DOI":"10.1002\/ana.24307","volume":"77","author":"M Dubey","year":"2015","unstructured":"Dubey M, Bugiani M, Ridder MC, Postma NL, Brouwers E, Polder E, Jacobs JG, Baayen JC, Klooster J, Kamermans M, et al. Mice with megalencephalic leukoencephalopathy with cysts: a developmental angle. Ann Neurol. 2015;77:114\u201331.","journal-title":"Ann Neurol"},{"key":"540_CR5","doi-asserted-by":"publisher","first-page":"450","DOI":"10.1002\/acn3.405","volume":"4","author":"M Bugiani","year":"2017","unstructured":"Bugiani M, Dubey M, Breur M, Postma NL, Dekker MP, Ter Braak T, Boschert U, Abbink TEM, Mansvelder HD, Min R, et al. Megalencephalic leukoencephalopathy with cysts: the Glialcam-null mouse model. Ann Clin Transl Neurol. 2017;4:450\u201365.","journal-title":"Ann Clin Transl Neurol"},{"key":"540_CR6","doi-asserted-by":"publisher","first-page":"3475","DOI":"10.1038\/ncomms4475","volume":"5","author":"MB Hoegg-Beiler","year":"2014","unstructured":"Hoegg-Beiler MB, Sirisi S, Orozco IJ, Ferrer I, Hohensee S, Auberson M, Godde K, Vilches C, de Heredia ML, Nunes V, et al. Disrupting MLC1 and GlialCAM and ClC-2 interactions in leukodystrophy entails glial chloride channel dysfunction. Nat Commun. 2014;5:3475.","journal-title":"Nat Commun"},{"key":"540_CR7","doi-asserted-by":"publisher","first-page":"264","DOI":"10.1002\/ana.10112","volume":"51","author":"MS van der Knaap","year":"2002","unstructured":"van der Knaap MS, Leegwater PA, Konst AA, Visser A, Naidu S, Oudejans CB, Schutgens RB, Pronk JC. Mutations in each of the five subunits of translation initiation factor eIF2B can cause leukoencephalopathy with vanishing white matter. Ann Neurol. 2002;51:264\u201370.","journal-title":"Ann Neurol"},{"key":"540_CR8","doi-asserted-by":"publisher","first-page":"117","DOI":"10.1038\/83679","volume":"27","author":"M Brenner","year":"2001","unstructured":"Brenner M, Johnson AB, Boespflug-Tanguy O, Rodriguez D, Goldman JE, Messing A. Mutations in GFAP, encoding glial fibrillary acidic protein, are associated with Alexander disease. Nat Genet. 2001;27:117\u201320.","journal-title":"Nat Genet"},{"key":"540_CR9","doi-asserted-by":"publisher","first-page":"442","DOI":"10.1177\/1073858412465655","volume":"19","author":"SC Barnett","year":"2013","unstructured":"Barnett SC, Linington C. Myelination: do astrocytes play a role? Neuroscientist. 2013;19:442\u201350.","journal-title":"Neuroscientist"},{"key":"540_CR10","doi-asserted-by":"publisher","first-page":"e1002146","DOI":"10.1371\/journal.pgen.1002146","volume":"7","author":"O Tress","year":"2011","unstructured":"Tress O, Maglione M, Zlomuzica A, May D, Dicke N, Degen J, Dere E, Kettenmann H, Hartmann D, Willecke K. Pathologic and phenotypic alterations in a mouse expressing a connexin47 missense mutation that causes Pelizaeus-Merzbacher-like disease in humans. PLoS Genet. 2011;7:e1002146.","journal-title":"PLoS Genet"},{"key":"540_CR11","doi-asserted-by":"publisher","first-page":"7743","DOI":"10.1523\/JNEUROSCI.0341-09.2009","volume":"29","author":"SE Lutz","year":"2009","unstructured":"Lutz SE, Zhao Y, Gulinello M, Lee SC, Raine CS, Brosnan CF. Deletion of astrocyte connexins 43 and 30 leads to a dysmyelinating phenotype and hippocampal CA1 vacuolation. J Neurosci. 2009;29:7743\u201352.","journal-title":"J Neurosci"},{"key":"540_CR12","doi-asserted-by":"crossref","unstructured":"Lanciotti A, Brignone MS, Bertini E, Petrucci TC, Aloisi F, Ambrosini E. Astrocytes: emerging stars in Leukodystrophy pathogenesis. Transl Neurosci. 2013;4:144-64.","DOI":"10.2478\/s13380-013-0118-1"},{"key":"540_CR13","doi-asserted-by":"publisher","first-page":"445","DOI":"10.1038\/nrm2720","volume":"10","author":"P Friedl","year":"2009","unstructured":"Friedl P, Gilmour D. Collective cell migration in morphogenesis, regeneration and cancer. Nat Rev Mol Cell Biol. 2009;10:445\u201357.","journal-title":"Nat Rev Mol Cell Biol"},{"key":"540_CR14","doi-asserted-by":"publisher","first-page":"3215","DOI":"10.1242\/jcs.036517","volume":"122","author":"CJ Weijer","year":"2009","unstructured":"Weijer CJ. Collective cell migration in development. J Cell Sci. 2009;122:3215\u201323.","journal-title":"J Cell Sci"},{"key":"540_CR15","doi-asserted-by":"publisher","first-page":"1009","DOI":"10.1083\/jcb.145.5.1009","volume":"145","author":"TM Svitkina","year":"1999","unstructured":"Svitkina TM, Borisy GG. Arp2\/3 complex and actin depolymerizing factor\/cofilin in dendritic organization and treadmilling of actin filament array in lamellipodia. J Cell Biol. 1999;145:1009\u201326.","journal-title":"J Cell Biol"},{"key":"540_CR16","doi-asserted-by":"publisher","first-page":"577","DOI":"10.1038\/nrm3861","volume":"15","author":"M Krause","year":"2014","unstructured":"Krause M, Gautreau A. Steering cell migration: lamellipodium dynamics and the regulation of directional persistence. Nat Rev Mol Cell Biol. 2014;15:577\u201390.","journal-title":"Nat Rev Mol Cell Biol"},{"key":"540_CR17","doi-asserted-by":"publisher","first-page":"1704","DOI":"10.1126\/science.1092053","volume":"302","author":"AJ Ridley","year":"2003","unstructured":"Ridley AJ, Schwartz MA, Burridge K, Firtel RA, Ginsberg MH, Borisy G, Parsons JT, Horwitz AR. Cell migration: integrating signals from front to back. Science. 2003;302:1704\u20139.","journal-title":"Science"},{"key":"540_CR18","doi-asserted-by":"publisher","first-page":"1012","DOI":"10.1016\/j.cell.2011.06.010","volume":"145","author":"AJ Ridley","year":"2011","unstructured":"Ridley AJ. Life at the leading edge. Cell. 2011;145:1012\u201322.","journal-title":"Cell"},{"key":"540_CR19","doi-asserted-by":"publisher","first-page":"713","DOI":"10.1038\/nrm2026","volume":"7","author":"ED Goley","year":"2006","unstructured":"Goley ED, Welch MD. The ARP2\/3 complex: an actin nucleator comes of age. Nat Rev Mol Cell Biol. 2006;7:713\u201326.","journal-title":"Nat Rev Mol Cell Biol"},{"key":"540_CR20","doi-asserted-by":"publisher","first-page":"451","DOI":"10.1146\/annurev.biophys.35.040405.101936","volume":"36","author":"TD Pollard","year":"2007","unstructured":"Pollard TD. Regulation of actin filament assembly by Arp2\/3 complex and formins. Annu Rev Biophys Biomol Struct. 2007;36:451\u201377.","journal-title":"Annu Rev Biophys Biomol Struct"},{"key":"540_CR21","doi-asserted-by":"publisher","first-page":"707","DOI":"10.1146\/annurev.biochem.77.060407.135452","volume":"79","author":"SB Padrick","year":"2010","unstructured":"Padrick SB, Rosen MK. Physical mechanisms of signal integration by WASP family proteins. Annu Rev Biochem. 2010;79:707\u201335.","journal-title":"Annu Rev Biochem"},{"key":"540_CR22","doi-asserted-by":"publisher","first-page":"237","DOI":"10.1038\/nrm2867","volume":"11","author":"KG Campellone","year":"2010","unstructured":"Campellone KG, Welch MD. A nucleator arms race: cellular control of actin assembly. Nat Rev Mol Cell Biol. 2010;11:237\u201351.","journal-title":"Nat Rev Mol Cell Biol"},{"key":"540_CR23","doi-asserted-by":"publisher","first-page":"1347","DOI":"10.1016\/S0960-9822(98)00015-3","volume":"8","author":"LM Machesky","year":"1998","unstructured":"Machesky LM, Insall RH. Scar1 and the related Wiskott-Aldrich syndrome protein, WASP, regulate the actin cytoskeleton through the Arp2\/3 complex. Curr Biol. 1998;8:1347\u201356.","journal-title":"Curr Biol"},{"key":"540_CR24","doi-asserted-by":"publisher","first-page":"7","DOI":"10.1038\/nrm3492","volume":"14","author":"JD Rotty","year":"2013","unstructured":"Rotty JD, Wu C, Bear JE. New insights into the regulation and cellular functions of the ARP2\/3 complex. Nat Rev Mol Cell Biol. 2013;14:7\u201312.","journal-title":"Nat Rev Mol Cell Biol"},{"key":"540_CR25","doi-asserted-by":"publisher","first-page":"370","DOI":"10.1016\/S0960-9822(01)00098-7","volume":"11","author":"AM Weaver","year":"2001","unstructured":"Weaver AM, Karginov AV, Kinley AW, Weed SA, Li Y, Parsons JT, Cooper JA. Cortactin promotes and stabilizes Arp2\/3-induced actin filament network formation. Curr Biol. 2001;11:370\u20134.","journal-title":"Curr Biol"},{"key":"540_CR26","doi-asserted-by":"publisher","first-page":"1270","DOI":"10.1016\/S0960-9822(02)01035-7","volume":"12","author":"AM Weaver","year":"2002","unstructured":"Weaver AM, Heuser JE, Karginov AV, Lee WL, Parsons JT, Cooper JA. Interaction of cortactin and N-WASp with Arp2\/3 complex. Curr Biol. 2002;12:1270\u20138.","journal-title":"Curr Biol"},{"key":"540_CR27","doi-asserted-by":"publisher","first-page":"3728","DOI":"10.1093\/hmg\/ddn269","volume":"17","author":"A Duarri","year":"2008","unstructured":"Duarri A, Teijido O, Lopez-Hernandez T, Scheper GC, Barriere H, Boor I, Aguado F, Zorzano A, Palacin M, Martinez A, et al. Molecular pathogenesis of megalencephalic leukoencephalopathy with subcortical cysts: mutations in MLC1 cause folding defects. Hum Mol Genet. 2008;17:3728\u201339.","journal-title":"Hum Mol Genet"},{"key":"540_CR28","doi-asserted-by":"publisher","first-page":"2581","DOI":"10.1093\/hmg\/ddh291","volume":"13","author":"O Teijido","year":"2004","unstructured":"Teijido O, Martinez A, Pusch M, Zorzano A, Soriano E, Del Rio JA, Palacin M, Estevez R. Localization and functional analyses of the MLC1 protein involved in megalencephalic leukoencephalopathy with subcortical cysts. Hum Mol Genet. 2004;13:2581\u201394.","journal-title":"Hum Mol Genet"},{"key":"540_CR29","doi-asserted-by":"publisher","first-page":"2166","DOI":"10.1093\/hmg\/dds032","volume":"21","author":"A Lanciotti","year":"2012","unstructured":"Lanciotti A, Brignone MS, Molinari P, Visentin S, De Nuccio C, Macchia G, Aiello C, Bertini E, Aloisi F, Petrucci TC, Ambrosini E. Megalencephalic leukoencephalopathy with subcortical cysts protein 1 functionally cooperates with the TRPV4 cation channel to activate the response of astrocytes to osmotic stress: dysregulation by pathological mutations. Hum Mol Genet. 2012;21:2166\u201380.","journal-title":"Hum Mol Genet"},{"key":"540_CR30","doi-asserted-by":"publisher","first-page":"973","DOI":"10.1016\/j.cell.2011.12.034","volume":"148","author":"C Wu","year":"2012","unstructured":"Wu C, Asokan SB, Berginski ME, Haynes EM, Sharpless NE, Griffith JD, Gomez SM, Bear JE. Arp2\/3 is critical for lamellipodia and response to extracellular matrix cues but is dispensable for chemotaxis. Cell. 2012;148:973\u201387.","journal-title":"Cell"},{"key":"540_CR31","doi-asserted-by":"publisher","first-page":"1208","DOI":"10.1126\/science.1175862","volume":"326","author":"TD Pollard","year":"2009","unstructured":"Pollard TD, Cooper JA. Actin, a central player in cell shape and movement. Science. 2009;326:1208\u201312.","journal-title":"Science"},{"key":"540_CR32","doi-asserted-by":"publisher","first-page":"1543","DOI":"10.1093\/hmg\/ddw032","volume":"25","author":"A Lanciotti","year":"2016","unstructured":"Lanciotti A, Brignone MS, Visentin S, De Nuccio C, Catacuzzeno L, Mallozzi C, Petrini S, Caramia M, Veroni C, Minnone G, et al. Megalencephalic leukoencephalopathy with subcortical cysts protein-1 regulates epidermal growth factor receptor signaling in astrocytes. Hum Mol Genet. 2016;25:1543\u201358.","journal-title":"Hum Mol Genet"},{"key":"540_CR33","doi-asserted-by":"publisher","first-page":"412","DOI":"10.1093\/jnen\/64.5.412","volume":"64","author":"PK Boor","year":"2005","unstructured":"Boor PK, de Groot K, Waisfisz Q, Kamphorst W, Oudejans CB, Powers JM, Pronk JC, Scheper GC, van der Knaap MS. MLC1: a novel protein in distal astroglial processes. J Neuropathol Exp Neurol. 2005;64:412\u20139.","journal-title":"J Neuropathol Exp Neurol"},{"key":"540_CR34","doi-asserted-by":"publisher","first-page":"307","DOI":"10.1016\/j.mcn.2013.07.001","volume":"56","author":"S Petrini","year":"2013","unstructured":"Petrini S, Minnone G, Coccetti M, Frank C, Aiello C, Cutarelli A, Ambrosini E, Lanciotti A, Brignone MS, D'Oria V, et al. Monocytes and macrophages as biomarkers for the diagnosis of megalencephalic leukoencephalopathy with subcortical cysts. Mol Cell Neurosci. 2013;56:307\u201321.","journal-title":"Mol Cell Neurosci"},{"key":"540_CR35","doi-asserted-by":"publisher","first-page":"581","DOI":"10.1016\/j.nbd.2009.11.008","volume":"37","author":"A Lanciotti","year":"2010","unstructured":"Lanciotti A, Brignone MS, Camerini S, Serafini B, Macchia G, Raggi C, Molinari P, Crescenzi M, Musumeci M, Sargiacomo M, et al. MLC1 trafficking and membrane expression in astrocytes: role of caveolin-1 and phosphorylation. Neurobiol Dis. 2010;37:581\u201395.","journal-title":"Neurobiol Dis"},{"key":"540_CR36","doi-asserted-by":"publisher","first-page":"3266","DOI":"10.1093\/hmg\/ddr238","volume":"20","author":"T Lopez-Hernandez","year":"2011","unstructured":"Lopez-Hernandez T, Sirisi S, Capdevila-Nortes X, Montolio M, Fernandez-Duenas V, Scheper GC, van der Knaap MS, Casquero P, Ciruela F, Ferrer I, et al. Molecular mechanisms of MLC1 and GLIALCAM mutations in megalencephalic leukoencephalopathy with subcortical cysts. Hum Mol Genet. 2011;20:3266\u201377.","journal-title":"Hum Mol Genet"},{"key":"540_CR37","doi-asserted-by":"publisher","first-page":"422","DOI":"10.1016\/j.ajhg.2011.02.009","volume":"88","author":"T Lopez-Hernandez","year":"2011","unstructured":"Lopez-Hernandez T, Ridder MC, Montolio M, Capdevila-Nortes X, Polder E, Sirisi S, Duarri A, Schulte U, Fakler B, Nunes V, et al. Mutant GlialCAM causes megalencephalic leukoencephalopathy with subcortical cysts, benign familial macrocephaly, and macrocephaly with retardation and autism. Am J Hum Genet. 2011;88:422\u201332.","journal-title":"Am J Hum Genet"},{"key":"540_CR38","doi-asserted-by":"publisher","first-page":"302","DOI":"10.1038\/sj.mp.4000869","volume":"6","author":"J Meyer","year":"2001","unstructured":"Meyer J, Huberth A, Ortega G, Syagailo YV, Jatzke S, Mossner R, Strom TM, Ulzheimer-Teuber I, Stober G, Schmitt A, Lesch KP. A missense mutation in a novel gene encoding a putative cation channel is associated with catatonic schizophrenia in a large pedigree. Mol Psychiatry. 2001;6:302\u20136.","journal-title":"Mol Psychiatry"},{"key":"540_CR39","doi-asserted-by":"publisher","first-page":"31","DOI":"10.1002\/ajmg.b.20115","volume":"125B","author":"M Kaganovich","year":"2004","unstructured":"Kaganovich M, Peretz A, Ritsner M, Bening Abu-Shach U, Attali B, Navon R. Is the WKL1 gene associated with schizophrenia? Am J Med Genet B Neuropsychiatr Genet. 2004;125B:31\u20137.","journal-title":"Am J Med Genet B Neuropsychiatr Genet"},{"key":"540_CR40","doi-asserted-by":"publisher","first-page":"90","DOI":"10.1093\/hmg\/ddq435","volume":"20","author":"MS Brignone","year":"2011","unstructured":"Brignone MS, Lanciotti A, Macioce P, Macchia G, Gaetani M, Aloisi F, Petrucci TC, Ambrosini E. The beta1 subunit of the Na,K-ATPase pump interacts with megalencephalic leucoencephalopathy with subcortical cysts protein 1 (MLC1) in brain astrocytes: new insights into MLC pathogenesis. Hum Mol Genet. 2011;20:90\u2013103.","journal-title":"Hum Mol Genet"},{"key":"540_CR41","doi-asserted-by":"publisher","first-page":"3342","DOI":"10.1093\/brain\/awr255","volume":"134","author":"MC Ridder","year":"2011","unstructured":"Ridder MC, Boor I, Lodder JC, Postma NL, Capdevila-Nortes X, Duarri A, Brussaard AB, Estevez R, Scheper GC, Mansvelder HD, van der Knaap MS. Megalencephalic leucoencephalopathy with cysts: defect in chloride currents and cell volume regulation. Brain. 2011;134:3342\u201354.","journal-title":"Brain"},{"key":"540_CR42","doi-asserted-by":"publisher","first-page":"88","DOI":"10.1016\/j.nbd.2018.07.031","volume":"119","author":"X Elorza-Vidal","year":"2018","unstructured":"Elorza-Vidal X, Sirisi S, Gaitan-Penas H, Perez-Rius C, Alonso-Gardon M, Armand-Ugon M, Lanciotti A, Brignone MS, Prat E, Nunes V, et al. GlialCAM\/MLC1 modulates LRRC8\/VRAC currents in an indirect manner: implications for megalencephalic leukoencephalopathy. Neurobiol Dis. 2018;119:88\u201399.","journal-title":"Neurobiol Dis"},{"key":"540_CR43","doi-asserted-by":"publisher","first-page":"1110","DOI":"10.1016\/j.neuron.2018.01.046","volume":"97","author":"M Mikhaylova","year":"2018","unstructured":"Mikhaylova M, Bar J, van Bommel B, Schatzle P, YuanXiang P, Raman R, Hradsky J, Konietzny A, Loktionov EY, Reddy PP, et al. Caldendrin directly couples postsynaptic calcium signals to actin remodeling in dendritic spines. Neuron. 2018;97:1110\u201325 e1114.","journal-title":"Neuron"},{"key":"540_CR44","doi-asserted-by":"publisher","first-page":"e19850","DOI":"10.7554\/eLife.19850","volume":"5","author":"P Wales","year":"2016","unstructured":"Wales P, Schuberth CE, Aufschnaiter R, Fels J, Garcia-Aguilar I, Janning A, Dlugos CP, Schafer-Herte M, Klingner C, Walte M, et al. Calcium-mediated actin reset (CaAR) mediates acute cell adaptations. Elife. 2016;5:e19850.","journal-title":"Elife"},{"key":"540_CR45","doi-asserted-by":"publisher","first-page":"890","DOI":"10.1083\/jcb.85.3.890","volume":"85","author":"KD McCarthy","year":"1980","unstructured":"McCarthy KD, de Vellis J. Preparation of separate astroglial and oligodendroglial cell cultures from rat cerebral tissue. J Cell Biol. 1980;85:890\u2013902.","journal-title":"J Cell Biol"},{"key":"540_CR46","doi-asserted-by":"crossref","unstructured":"Schildge S, Bohrer C, Beck K, Schachtrup C. Isolation and culture of mouse cortical astrocytes. J Vis Exp. 2013;71:50079.","DOI":"10.3791\/50079"},{"key":"540_CR47","doi-asserted-by":"publisher","first-page":"228","DOI":"10.1016\/j.nbd.2011.03.015","volume":"43","author":"A Duarri","year":"2011","unstructured":"Duarri A, Lopez de Heredia M, Capdevila-Nortes X, Ridder MC, Montolio M, Lopez-Hernandez T, Boor I, Lien CF, Hagemann T, Messing A, et al. Knockdown of MLC1 in primary astrocytes causes cell vacuolation: a MLC disease cell model. Neurobiol Dis. 2011;43:228\u201338.","journal-title":"Neurobiol Dis"}],"container-title":["Molecular Brain"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13041-019-0540-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1186\/s13041-019-0540-6\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s13041-019-0540-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,12,29]],"date-time":"2020-12-29T00:15:38Z","timestamp":1609200938000},"score":1,"resource":{"primary":{"URL":"https:\/\/molecularbrain.biomedcentral.com\/articles\/10.1186\/s13041-019-0540-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,12]]},"references-count":47,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2019,12]]}},"alternative-id":["540"],"URL":"https:\/\/doi.org\/10.1186\/s13041-019-0540-6","relation":{},"ISSN":["1756-6606"],"issn-type":[{"value":"1756-6606","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,12]]},"assertion":[{"value":"12 November 2019","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"18 December 2019","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"30 December 2019","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"All procedures were performed following protocols approved by the Animal Care and Use Committee of Korea Brain Research Institute (IACUC-2018-0013).","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare they have no competing interests.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"116"}}