{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T15:23:19Z","timestamp":1791991399626,"version":"4.4.0"},"reference-count":81,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2017,4,1]],"date-time":"2017-04-01T00:00:00Z","timestamp":1491004800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/501100001543","name":"New Zealand Neurological Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100001543","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Otago School of Medical Sciences Dean's Bequest Funds"},{"name":"Brain Health Research Centre, NZ"}],"content-domain":{"domain":["clinicalkey.jp","clinicalkey.com","clinicalkey.es","clinicalkey.com.au","clinicalkey.fr","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neurobiology of Disease"],"published-print":{"date-parts":[[2017,4]]},"DOI":"10.1016\/j.nbd.2017.01.001","type":"journal-article","created":{"date-parts":[[2017,1,6]],"date-time":"2017-01-06T04:29:01Z","timestamp":1483676941000},"page":"62-74","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":33,"special_numbering":"C","title":["Characterisation of early changes in ovine CLN5 and CLN6 Batten disease neural cultures for the rapid screening of therapeutics"],"prefix":"10.1016","volume":"100","author":[{"given":"Hannah L","family":"Best","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole J","family":"Neverman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hollie E","family":"Wicky","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadia L","family":"Mitchell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Beulah","family":"Leitch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephanie M","family":"Hughes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.nbd.2017.01.001_bb0005","doi-asserted-by":"crossref","DOI":"10.1002\/brb3.401","article-title":"Molecular neuropathology of the synapse in sheep with CLN5 Batten disease","volume":"5","author":"Amorim","year":"2015","journal-title":"Brain Behav."},{"key":"10.1016\/j.nbd.2017.01.001_bb0010","doi-asserted-by":"crossref","first-page":"6952","DOI":"10.1167\/iovs.13-12945","article-title":"Apoptotic photoreceptor loss and altered expression of lysosomal proteins in the nclf mouse model of neuronal ceroid lipofuscinosis","volume":"54","author":"Bartsch","year":"2013","journal-title":"Invest. Ophthalmol. Vis. Sci."},{"key":"10.1016\/j.nbd.2017.01.001_bb0015","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1146\/annurev-biochem-060815-014556","article-title":"Mammalian autophagy: how does it work?","volume":"85","author":"Bento","year":"2016","journal-title":"Annu. Rev. Biochem."},{"key":"10.1016\/j.nbd.2017.01.001_bb0020","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1016\/j.ymeth.2014.11.013","article-title":"The versatile electron microscope: an ultrastructural overview of autophagy","volume":"75","author":"Biazik","year":"2015","journal-title":"Methods"},{"key":"10.1016\/j.nbd.2017.01.001_bb0025","doi-asserted-by":"crossref","first-page":"1842","DOI":"10.1016\/j.bbadis.2013.01.009","article-title":"Use of model organisms for the study of neuronal ceroid lipofuscinosis","volume":"1832","author":"Bond","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.nbd.2017.01.001_bb0030","doi-asserted-by":"crossref","first-page":"9669","DOI":"10.1523\/JNEUROSCI.1635-16.2016","article-title":"Self-complementary AAV9 gene delivery partially corrects pathology associated with juvenile neuronal ceroid lipofuscinosis (CLN3)","volume":"36","author":"Bosch","year":"2016","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2017.01.001_bb0035","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1093\/hmg\/ddv615","article-title":"Lysosomal dysfunction and impaired autophagy in a novel mouse model deficient for the lysosomal membrane protein Cln7","volume":"25","author":"Brandenstein","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2017.01.001_bb0040","doi-asserted-by":"crossref","first-page":"20483","DOI":"10.1074\/jbc.M602180200","article-title":"Autophagy is disrupted in a knock-in mouse model of juvenile neuronal ceroid lipofuscinosis","volume":"281","author":"Cao","year":"2006","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2017.01.001_bb0045","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0017118","article-title":"Distinct early molecular responses to mutations causing vLINCL and JNCL presage ATP synthase subunit C accumulation in cerebellar cells","volume":"6","author":"Cao","year":"2011","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2017.01.001_bb0050","doi-asserted-by":"crossref","DOI":"10.1038\/ncomms9620","article-title":"Pharmaceutical screen identifies novel target processes for activation of autophagy with a broad translational potential","volume":"6","author":"Chauhan","year":"2015","journal-title":"Nat. Commun."},{"key":"10.1016\/j.nbd.2017.01.001_bb0055","doi-asserted-by":"crossref","first-page":"8151","DOI":"10.1523\/JNEUROSCI.0293-10.2010","article-title":"Activity-dependent bulk endocytosis and clathrin-dependent endocytosis replenish specific synaptic vesicle pools in central nerve terminals","volume":"30","author":"Cheung","year":"2010","journal-title":"J. Neurosci."},{"key":"10.1016\/j.nbd.2017.01.001_bb0060","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/j.jneumeth.2009.09.016","article-title":"Quantitative monitoring of activity-dependent bulk endocytosis of synaptic vesicle membrane by fluorescent dextran imaging","volume":"185","author":"Clayton","year":"2009","journal-title":"J. Neurosci. Methods"},{"key":"10.1016\/j.nbd.2017.01.001_bb0065","doi-asserted-by":"crossref","first-page":"1448","DOI":"10.1042\/BST0381448","article-title":"The neuronal ceroid lipofuscinoses: the same, but different?","volume":"38","author":"Cooper","year":"2010","journal-title":"Biochem. Soc. Trans."},{"key":"10.1016\/j.nbd.2017.01.001_bb0070","doi-asserted-by":"crossref","first-page":"2709","DOI":"10.1093\/hmg\/11.22.2709","article-title":"Cln3 (Deltaex7\/8) knock-in mice with the common JNCL mutation exhibit progressive neurologic disease that begins before birth","volume":"11","author":"Cotman","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2017.01.001_bb0075","doi-asserted-by":"crossref","first-page":"13013","DOI":"10.1038\/srep13013","article-title":"Rhus coriaria induces senescence and autophagic cell death in breast cancer cells through a mechanism involving p38 and ERK1\/2 activation","volume":"5","author":"El Hasasna","year":"2015","journal-title":"Sci. Rep."},{"key":"10.1016\/j.nbd.2017.01.001_bb0080","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1186\/1471-2202-5-57","article-title":"Membrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosis","volume":"5","author":"Fossale","year":"2004","journal-title":"BMC Neurosci."},{"key":"10.1016\/j.nbd.2017.01.001_bb0085","doi-asserted-by":"crossref","first-page":"306","DOI":"10.1016\/j.nbd.2007.09.006","article-title":"A new large animal model of CLN5 neuronal ceroid lipofuscinosis in Borderdale sheep is caused by a nucleotide substitution at a consensus splice site (c.571+1G>A) leading to excision of exon 3","volume":"29","author":"Frugier","year":"2008","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2017.01.001_bb0090","doi-asserted-by":"crossref","first-page":"38922","DOI":"10.1074\/jbc.M112.365148","article-title":"Gemfibrozil and fenofibrate, Food and Drug Administration-approved lipidlowering drugs, up-regulate tripeptidyl-peptidase 1 in brain cells via peroxisome proliferatoractivated receptor alpha: implications for late infantile Batten disease therapy","volume":"287","author":"Ghosh","year":"2012","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2017.01.001_bb0095","doi-asserted-by":"crossref","first-page":"10309","DOI":"10.1074\/jbc.M114.610659","article-title":"Activation of peroxisome proliferator-activated receptor alpha induces lysosomal biogenesis in brain cells: implications for lysosomal storage disorders","volume":"290","author":"Ghosh","year":"2015","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2017.01.001_bb0100","doi-asserted-by":"crossref","first-page":"829","DOI":"10.1038\/sj.embor.7401042","article-title":"A multiplicity of cell death pathways. Symposium on apoptotic and non-apoptotic cell death pathways","volume":"8","author":"Golstein","year":"2007","journal-title":"EMBO Rep."},{"key":"10.1016\/j.nbd.2017.01.001_bb0105","doi-asserted-by":"crossref","first-page":"560","DOI":"10.1080\/15548627.2015.1017181","article-title":"A rapid and high content assay that measures cyto-ID-stained autophagic compartments and estimates autophagy flux with potential clinical applications","volume":"11","author":"Guo","year":"2015","journal-title":"Autophagy"},{"key":"10.1016\/j.nbd.2017.01.001_bb0110","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1038\/nature04724","article-title":"Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice","volume":"441","author":"Hara","year":"2006","journal-title":"Nature"},{"key":"10.1016\/j.nbd.2017.01.001_bb0115","doi-asserted-by":"crossref","first-page":"881","DOI":"10.1084\/jem.174.4.881","article-title":"Ammonium chloride, an inhibitor of phagosome-lysosome fusion in macrophages, concurrently induces phagosome-endosome fusion, and opens a novel pathway: studies of a pathogenic mycobacterium and a nonpathogenic yeast","volume":"174","author":"Hart","year":"1991","journal-title":"J. Exp. Med."},{"key":"10.1016\/j.nbd.2017.01.001_bb0120","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1038\/sj.gt.3301843","article-title":"Viral-mediated delivery of the late-infantile neuronal ceroid lipofuscinosis gene, TPP-I to the mouse central nervous system","volume":"10","author":"Haskell","year":"2003","journal-title":"Gene Ther."},{"key":"10.1016\/j.nbd.2017.01.001_bb0125","doi-asserted-by":"crossref","first-page":"22347","DOI":"10.1074\/jbc.M400643200","article-title":"Defective endoplasmic reticulum-resident membrane protein CLN6 affects lysosomal degradation of endocytosed arylsulfatase A","volume":"279","author":"Heine","year":"2004","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2017.01.001_bb0130","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1080\/09687860600967317","article-title":"Topology and endoplasmic reticulum retention signals of the lysosomal storage disease-related membrane protein CLN6","volume":"24","author":"Heine","year":"2007","journal-title":"Mol. Membr. Biol."},{"key":"10.1016\/j.nbd.2017.01.001_bb0135","series-title":"BioTek Instruments Application Guides","article-title":"Monitoring viral infection of mammalian cells using digital fluorescence microscopy","author":"Held","year":"2016"},{"key":"10.1016\/j.nbd.2017.01.001_bb0140","doi-asserted-by":"crossref","first-page":"5851","DOI":"10.1046\/j.0014-2956.2001.02530.x","article-title":"Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)","volume":"268","author":"Holopainen","year":"2001","journal-title":"Eur. J. Biochem."},{"issue":"3","key":"10.1016\/j.nbd.2017.01.001_bb0150","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1007\/s11626-015-9979-7","article-title":"Fibrates inhibit the apoptosis of Batten disease lymphoblast cells via autophagy recovery and regulation of mitochondrial membrane potential","volume":"52","author":"Hong","year":"2015","journal-title":"In Vitro Cell. Dev. Biol. Anim."},{"key":"10.1016\/j.nbd.2017.01.001_bb0155","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1016\/j.nbd.2013.11.011","article-title":"Inhibition of storage pathology in prenatal CLN5-deficient sheep neural cultures by lentiviral gene therapy","volume":"62","author":"Hughes","year":"2014","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.nbd.2017.01.001_bb0160","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1093\/hmg\/11.8.885","article-title":"Lysosomal localization of the neuronal ceroid lipofuscinosis CLN5 protein","volume":"11","author":"Isosomppi","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2017.01.001_bb0165","article-title":"Microglia actively regulate the number of functional synapses","volume":"8","author":"Ji","year":"2013","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2017.01.001_bb0170","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1080\/00480169.1976.34298","article-title":"Blindness in South Hampshire sheep: a neuronal ceroidlipofuscinosis","volume":"24","author":"Jolly","year":"1976","journal-title":"N. Z. Vet. J."},{"key":"10.1016\/j.nbd.2017.01.001_bb0175","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1080\/00480169.2002.36311","article-title":"Neuronal ceroid-lipofuscinosis in Borderdale sheep","volume":"50","author":"Jolly","year":"2002","journal-title":"N. Z. Vet. J."},{"key":"10.1016\/j.nbd.2017.01.001_bb0180","doi-asserted-by":"crossref","DOI":"10.1126\/scitranslmed.aac6191","article-title":"AAV gene transfer delays disease onset in a TPP1-deficient canine model of the late infantile form of Batten disease","volume":"7","author":"Katz","year":"2015","journal-title":"Sci. Transl. Med."},{"key":"10.1016\/j.nbd.2017.01.001_bb0185","doi-asserted-by":"crossref","first-page":"4066","DOI":"10.1093\/hmg\/ddp355","article-title":"Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease","volume":"18","author":"Kielar","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2017.01.001_bb0190","doi-asserted-by":"crossref","first-page":"549","DOI":"10.4161\/auto.28448","article-title":"Autophagosomes, phagosomes, autolysosomes, phagolysosomes, autophagolysosomes\u2026 wait, I'm confused","volume":"10","author":"Klionsky","year":"2014","journal-title":"Autophagy"},{"key":"10.1016\/j.nbd.2017.01.001_bb0195","doi-asserted-by":"crossref","first-page":"1713","DOI":"10.1016\/S0002-9440(10)61253-9","article-title":"Participation of autophagy in storage of lysosomes in neurons from mouse models of neuronal ceroid-lipofuscinoses (Batten disease)","volume":"167","author":"Koike","year":"2005","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.nbd.2017.01.001_bb0200","doi-asserted-by":"crossref","first-page":"4263","DOI":"10.1002\/humu.21624","article-title":"Update of the mutation spectrum and clinical correlations of over 360 mutations in eight genes that underlie the neuronal ceroid lipofuscinoses","volume":"33","author":"Kousi","year":"2012","journal-title":"Hum. Mutat."},{"issue":"Suppl. 2","key":"10.1016\/j.nbd.2017.01.001_bb0205","doi-asserted-by":"crossref","first-page":"1463","DOI":"10.1038\/sj.cdd.4401724","article-title":"Classification of cell death: recommendations of the Nomenclature Committee on Cell Death","volume":"12","author":"Kroemer","year":"2005","journal-title":"Cell Death Differ."},{"key":"10.1016\/j.nbd.2017.01.001_bb0210","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1016\/0022-510X(78)90087-4","article-title":"Early-juvenile Battens disease - recognizable subgroup distinct from other forms of Batten disease - analysis of 5 patients","volume":"36","author":"Lake","year":"1978","journal-title":"J. Neurol. Sci."},{"key":"10.1016\/j.nbd.2017.01.001_bb0215","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1172\/JCI73938","article-title":"Development of autophagy inducers in clinical medicine","volume":"125","author":"Levine","year":"2015","journal-title":"J. Clin. Investig."},{"key":"10.1016\/j.nbd.2017.01.001_bb0220","doi-asserted-by":"crossref","first-page":"1011","DOI":"10.1089\/hum.2011.026","article-title":"Lentiviral-mediated gene transfer to the sheep brain: implications for gene therapy in Batten disease","volume":"22","author":"Linterman","year":"2011","journal-title":"Hum. Gene Ther."},{"key":"10.1016\/j.nbd.2017.01.001_bb0225","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1089\/104303403764539387","article-title":"Real-time quantitative reverse transcriptase-polymerase chain reaction as a method for determining lentiviral vector titers and measuring transgene expression","volume":"14","author":"Lizee","year":"2003","journal-title":"Hum. Gene Ther."},{"key":"10.1016\/j.nbd.2017.01.001_bb0230","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1038\/nbt1186","article-title":"Statistical practice in high-throughput screening data analysis","volume":"24","author":"Malo","year":"2006","journal-title":"Nat. Biotechnol."},{"key":"10.1016\/j.nbd.2017.01.001_bb0235","doi-asserted-by":"crossref","first-page":"1855","DOI":"10.1128\/MCB.06726-11","article-title":"The role of ceroid lipofuscinosis neuronal protein 5 (CLN5) in endosomal sorting","volume":"32","author":"Mamo","year":"2012","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.nbd.2017.01.001_bb0240","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1016\/j.tins.2016.02.002","article-title":"The autophagy-lysosomal pathway in neurodegeneration: a TFEB perspective","volume":"39","author":"Martini-Stoica","year":"2016","journal-title":"Trends Neurosci."},{"key":"10.1016\/j.nbd.2017.01.001_bb0245","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1038\/nrn3961","article-title":"Compromised autophagy and neurodegenerative diseases","volume":"16","author":"Menzies","year":"2015","journal-title":"Nat. Rev. Neurosci."},{"key":"10.1016\/j.nbd.2017.01.001_bb0250","doi-asserted-by":"crossref","first-page":"1128","DOI":"10.1128\/JVI.01229-07","article-title":"Inhibition of endosome-lysosome system acidification enhances porcine circovirus 2 infection of porcine epithelial cells","volume":"82","author":"Misinzo","year":"2008","journal-title":"J. Virol."},{"key":"10.1016\/j.nbd.2017.01.001_bb0255","doi-asserted-by":"crossref","first-page":"2237","DOI":"10.1016\/j.bbadis.2015.05.011","article-title":"Genetics of the neuronal ceroid lipofuscinoses (Batten disease)","volume":"1852","author":"Mole","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.nbd.2017.01.001_bb0260","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1016\/j.yexcr.2004.04.042","article-title":"CLN6, which is associated with a lysosomal storage disease, is an endoplasmic reticulum protein","volume":"298","author":"Mole","year":"2004","journal-title":"Exp. Cell Res."},{"key":"10.1016\/j.nbd.2017.01.001_bb0265","doi-asserted-by":"crossref","first-page":"2292","DOI":"10.1016\/j.bbadis.2015.04.026","article-title":"Experimental therapies in the neuronal ceroid lipofuscinoses","volume":"1852","author":"Neverman","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.nbd.2017.01.001_bb0270","doi-asserted-by":"crossref","first-page":"9220","DOI":"10.1128\/MCB.01453-06","article-title":"Autophagy is activated for cell survival after endoplasmic reticulum stress","volume":"26","author":"Ogata","year":"2006","journal-title":"Mol. Cell. Biol."},{"issue":"Suppl. 5A","key":"10.1016\/j.nbd.2017.01.001_bb0275","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1053\/ejpn.2000.0450","article-title":"Changes in GABAergic neuron distribution in situ and in neuron cultures in ovine (OCL6) Batten disease","author":"Oswald","year":"2001","journal-title":"Eur. J. Paediatr. Neurol."},{"key":"10.1016\/j.nbd.2017.01.001_bb0280","doi-asserted-by":"crossref","first-page":"1165","DOI":"10.1007\/s00018-005-5406-7","article-title":"Lipid-lowering drugs","volume":"63","author":"Pahan","year":"2006","journal-title":"Cell. Mol. Life Sci."},{"issue":"10","key":"10.1016\/j.nbd.2017.01.001_bb0285","doi-asserted-by":"crossref","first-page":"5736","DOI":"10.1016\/S0021-9258(18)83610-9","article-title":"Ovine ceroid lipofuscinosis. The major lipopigment protein and the lipid-binding subunit of mitochondrial ATP synthase have the same NH2-terminal sequence","volume":"264","author":"Palmer","year":"1989","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2017.01.001_bb0290","doi-asserted-by":"crossref","first-page":"2279","DOI":"10.1016\/j.bbadis.2015.06.013","article-title":"Recent studies of ovine neuronal ceroid lipofuscinoses from BARN, the Batten Animal Research Network","volume":"1852","author":"Palmer","year":"2015","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.nbd.2017.01.001_bb0295","doi-asserted-by":"crossref","first-page":"3852","DOI":"10.1093\/hmg\/ddr306","article-title":"Characterization of the CLEAR network reveals an integrated control of cellular clearance pathways","volume":"20","author":"Palmieri","year":"2011","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2017.01.001_bb0300","doi-asserted-by":"crossref","first-page":"1085","DOI":"10.1002\/acn3.261","article-title":"Suppression of agrin-22 production and synaptic dysfunction in Cln1 (\u2212\/\u2212) mice","volume":"2","author":"Peng","year":"2015","journal-title":"Ann. Clin. Transl. Neurol."},{"key":"10.1016\/j.nbd.2017.01.001_bb0305","doi-asserted-by":"crossref","DOI":"10.1093\/nar\/29.9.e45","article-title":"A new mathematical model for relative quantification in real-time RT-PCR","volume":"29","author":"Pfaffl","year":"2001","journal-title":"Nucleic Acids Res."},{"key":"10.1016\/j.nbd.2017.01.001_bb0310","doi-asserted-by":"crossref","first-page":"2177","DOI":"10.1111\/j.1471-4159.2007.04920.x","article-title":"Increased expression of lysosomal acid phosphatase in CLN3-defective cells and mouse brain tissue","volume":"103","author":"Pohl","year":"2007","journal-title":"J. Neurochem."},{"key":"10.1016\/j.nbd.2017.01.001_bb0315","doi-asserted-by":"crossref","first-page":"1142","DOI":"10.15252\/emmm.201303671","article-title":"Selective clearance of aberrant tau proteins and rescue of neurotoxicity by transcription factor EB","volume":"6","author":"Polito","year":"2014","journal-title":"EMBO Mol. Med."},{"key":"10.1016\/j.nbd.2017.01.001_bb0320","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1186\/s13041-014-0063-0","article-title":"Human primary mixed brain cultures: preparation, differentiation, characterization and application to neuroscience research","volume":"7","author":"Ray","year":"2014","journal-title":"Mol. Brain"},{"key":"10.1016\/j.nbd.2017.01.001_bb0325","doi-asserted-by":"crossref","first-page":"11061","DOI":"10.1074\/jbc.R109.072181","article-title":"Chemical inducers of autophagy that enhance the clearance of mutant proteins in neurodegenerative diseases","volume":"285","author":"Renna","year":"2010","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2017.01.001_bb0330","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1007\/s10867-009-9157-9","article-title":"Astrocyte-neuron interactions in neurological disorders","volume":"35","author":"Ricci","year":"2009","journal-title":"J. Biol. Phys."},{"key":"10.1016\/j.nbd.2017.01.001_bb0335","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1002\/ajmg.1320310606","article-title":"Batten disease: past, present, and future","volume":"5","author":"Rider","year":"1988","journal-title":"Am. J. Med. Genet. Suppl."},{"key":"10.1016\/j.nbd.2017.01.001_bb0340","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1016\/S1534-5807(02)00145-4","article-title":"GPI-anchored proteins are delivered to recycling endosomes via a distinct cdc42-regulated, clathrin-independent pinocytic pathway","volume":"2","author":"Sabharanjak","year":"2002","journal-title":"Dev. Cell"},{"key":"10.1016\/j.nbd.2017.01.001_bb0345","doi-asserted-by":"crossref","first-page":"4021","DOI":"10.4161\/cc.8.24.10263","article-title":"Lysosomal enhancement: a CLEAR answer to cellular degradative needs","volume":"8","author":"Sardiello","year":"2009","journal-title":"Cell Cycle"},{"key":"10.1016\/j.nbd.2017.01.001_bb0350","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1038\/gt.2015.3","article-title":"Chimeric rabies SADB19-VSVg-pseudotyped lentiviral vectors mediate long-range retrograde transduction from the mouse spinal cord","volume":"22","author":"Schoderboeck","year":"2015","journal-title":"Gene Ther."},{"key":"10.1016\/j.nbd.2017.01.001_bb0355","doi-asserted-by":"crossref","first-page":"14291433","DOI":"10.1126\/science.1204592","article-title":"TFEB links autophagy to lysosomal biogenesis","volume":"332","author":"Settembre","year":"2011","journal-title":"Science"},{"key":"10.1016\/j.nbd.2017.01.001_bb0360","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.conb.2016.03.005","article-title":"Modes and mechanisms of synaptic vesicle recycling","volume":"39","author":"Soykan","year":"2016","journal-title":"Curr. Opin. Neurobiol."},{"key":"10.1016\/j.nbd.2017.01.001_bb0365","doi-asserted-by":"crossref","first-page":"898","DOI":"10.1016\/j.bbadis.2006.09.004","article-title":"A missense mutation (c.184C>T) in ovine CLN6 causes neuronal ceroid lipofuscinosis in Merino sheep whereas affected South Hampshire sheep have reduced levels of CLN6 mRNA","volume":"1762","author":"Tammen","year":"2006","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.nbd.2017.01.001_bb0370","doi-asserted-by":"crossref","DOI":"10.1371\/annotation\/a4b06d46-8eb9-4d15-a15a-41bf4b5ccb8b","article-title":"Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis","volume":"7","author":"Thelen","year":"2012","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2017.01.001_bb0375","doi-asserted-by":"crossref","first-page":"15765","DOI":"10.1007\/s12038-011-9176-0","article-title":"Autophagy: a double-edged sword in Alzheimer's disease","volume":"37","author":"Tung","year":"2012","journal-title":"J. Biosci."},{"key":"10.1016\/j.nbd.2017.01.001_bb0380","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0055526","article-title":"Alterations in ROS activity and lysosomal pH account for distinct patterns of macroautophagy in LINCL and JNCL fibroblasts","volume":"8","author":"Vidal-Donet","year":"2013","journal-title":"PLoS One"},{"key":"10.1016\/j.nbd.2017.01.001_bb0385","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1186\/1471-2164-9-146","article-title":"Brain gene expression profiles of Cln1 and Cln5 deficient mice unravels common molecular pathways underlying neuronal degeneration in NCL diseases","volume":"9","author":"Von Schantz","year":"2008","journal-title":"BMC Genomics"},{"key":"10.1016\/j.nbd.2017.01.001_bb0390","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1089\/hum.2008.022","article-title":"Treatment of late infantile neuronal ceroid lipofuscinosis by CNS administration of a serotype 2 adeno-associated virus expressing CLN2 cDNA","volume":"19","author":"Worgall","year":"2008","journal-title":"Hum. Gene Ther."},{"issue":"5","key":"10.1016\/j.nbd.2017.01.001_bb0395","doi-asserted-by":"crossref","first-page":"1815","DOI":"10.1172\/JCI82409","article-title":"Amyloid precursor protein-mediated endocytic pathway disruption induces axonal dysfunction and neurodegeneration","volume":"126","author":"Xu","year":"2016","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.nbd.2017.01.001_bb0400","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1007\/BF02531798","article-title":"What is amaurotic idiocy","volume":"4","author":"Zeman","year":"1969","journal-title":"Lipids"},{"key":"10.1016\/j.nbd.2017.01.001_bb0405","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1177\/108705719900400206","article-title":"A simple statistical parameter for use in evaluation and validation of high throughput screening assays","volume":"4","author":"Zhang","year":"1999","journal-title":"J. Biomol. Screen."},{"key":"10.1016\/j.nbd.2017.01.001_bb0410","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1038\/nbt0997-871","article-title":"Multiply attenuated lentiviral vector achieves efficient gene delivery in vivo","volume":"15","author":"Zufferey","year":"1997","journal-title":"Nat. Biotechnol."}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996117300013?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996117300013?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2022,7,20]],"date-time":"2022-07-20T21:08:17Z","timestamp":1658351297000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0969996117300013"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,4]]},"references-count":81,"alternative-id":["S0969996117300013"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2017.01.001","relation":{},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2017,4]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Characterisation of early changes in ovine CLN5 and CLN6 Batten disease neural cultures for the rapid screening of therapeutics","name":"articletitle","label":"Article Title"},{"value":"Neurobiology of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.nbd.2017.01.001","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2017 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}