{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,17]],"date-time":"2026-08-17T19:23:41Z","timestamp":1786994621804,"version":"build-2736575974"},"reference-count":72,"publisher":"Elsevier BV","issue":"1","license":[{"start":{"date-parts":[[2012,4,1]],"date-time":"2012-04-01T00:00:00Z","timestamp":1333238400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2012,4,1]],"date-time":"2012-04-01T00:00:00Z","timestamp":1333238400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2012,4,1]],"date-time":"2012-04-01T00:00:00Z","timestamp":1333238400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2012,4,1]],"date-time":"2012-04-01T00:00:00Z","timestamp":1333238400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2012,4,1]],"date-time":"2012-04-01T00:00:00Z","timestamp":1333238400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2012,4,1]],"date-time":"2012-04-01T00:00:00Z","timestamp":1333238400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2012,4,1]],"date-time":"2012-04-01T00:00:00Z","timestamp":1333238400000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Neurobiology of Disease"],"published-print":{"date-parts":[[2012,4]]},"DOI":"10.1016\/j.nbd.2011.12.009","type":"journal-article","created":{"date-parts":[[2011,12,13]],"date-time":"2011-12-13T14:24:44Z","timestamp":1323786284000},"page":"19-29","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":48,"title":["Cln5-deficiency in mice leads to microglial activation, defective myelination and changes in lipid metabolism"],"prefix":"10.1016","volume":"46","author":[{"given":"Mia-Lisa","family":"Schmiedt","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tea","family":"Blom","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomas","family":"Blom","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Outi","family":"Kopra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew","family":"Wong","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carina","family":"von Schantz-Fant","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elina","family":"Ikonen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mervi","family":"Kuronen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matti","family":"Jauhiainen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan D.","family":"Cooper","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anu","family":"Jalanko","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"1","key":"10.1016\/j.nbd.2011.12.009_bb0005","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1016\/j.nbd.2007.06.012","article-title":"Palmitoyl protein thioesterase 1 (Ppt1)-deficient mouse neurons show alterations in cholesterol metabolism and calcium homeostasis prior to synaptic dysfunction","volume":"28","author":"Ahtiainen","year":"2007","journal-title":"Neurobiol. Dis."},{"issue":"2","key":"10.1016\/j.nbd.2011.12.009_bb0010","doi-asserted-by":"crossref","first-page":"346","DOI":"10.1016\/j.nbd.2004.02.010","article-title":"Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis","volume":"16","author":"Bible","year":"2004","journal-title":"Neurobiol. Dis."},{"issue":"4","key":"10.1016\/j.nbd.2011.12.009_bb0015","doi-asserted-by":"crossref","first-page":"720","DOI":"10.1161\/CIRCRESAHA.109.204396","article-title":"FTY720 stimulates 27-hydroxycholesterol production and confers atheroprotective effects in human primary macrophages","volume":"106","author":"Blom","year":"2010","journal-title":"Circ. Res."},{"issue":"2","key":"10.1016\/j.nbd.2011.12.009_bb0020","doi-asserted-by":"crossref","first-page":"144","DOI":"10.1097\/00005072-199403000-00005","article-title":"Immunohistochemical detection of myelin basic protein is a sensitive marker of myelination in second trimester human fetal spinal cord","volume":"53","author":"Bodhireddy","year":"1994","journal-title":"J. Neuropathol. Exp. Neurol."},{"issue":"17","key":"10.1016\/j.nbd.2011.12.009_bb0025","doi-asserted-by":"crossref","first-page":"1945","DOI":"10.1007\/s00018-006-6094-7","article-title":"Myelin basic protein: a multifunctional protein","volume":"63","author":"Boggs","year":"2006","journal-title":"Cell. Mol. Life Sci."},{"issue":"2","key":"10.1016\/j.nbd.2011.12.009_bb0030","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1007\/s00401-007-0296-4","article-title":"Immune system irregularities in lysosomal storage disorders","volume":"115","author":"Castaneda","year":"2008","journal-title":"Acta Neuropathol."},{"issue":"11","key":"10.1016\/j.nbd.2011.12.009_bb0035","doi-asserted-by":"crossref","first-page":"6373","DOI":"10.1073\/pnas.95.11.6373","article-title":"Abnormal transport along the lysosomal pathway in mucolipidosis, type IV disease","volume":"95","author":"Chen","year":"1998","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"issue":"9182","key":"10.1016\/j.nbd.2011.12.009_bb0040","doi-asserted-by":"crossref","first-page":"901","DOI":"10.1016\/S0140-6736(98)10034-X","article-title":"Broad screening test for sphingolipid-storage diseases","volume":"354","author":"Chen","year":"1999","journal-title":"Lancet"},{"issue":"6","key":"10.1016\/j.nbd.2011.12.009_bb0045","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."},{"issue":"Suppl. A","key":"10.1016\/j.nbd.2011.12.009_bb0365","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1053\/ejpn.2000.0451","article-title":"Altered levels of high-energy phosphate compounds in fibroblasts from different forms of neuronal ceroid lipofuscinoses: further evidence for mitochondrial involvement","volume":"5","author":"Das","year":"2001","journal-title":"Eur. J. Paediatr. Neurol."},{"issue":"8","key":"10.1016\/j.nbd.2011.12.009_bb0055","doi-asserted-by":"crossref","first-page":"1375","DOI":"10.1194\/jlr.R400004-JLR200","article-title":"Thematic review series: brain Lipids. Cholesterol metabolism in the central nervous system during early development and in the mature animal","volume":"45","author":"Dietschy","year":"2004","journal-title":"J. Lipid Res."},{"issue":"4","key":"10.1016\/j.nbd.2011.12.009_bb0060","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1161\/01.ATV.21.4.473","article-title":"Paraoxonase and atherosclerosis","volume":"21","author":"Durrington","year":"2001","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"key":"10.1016\/j.nbd.2011.12.009_bb0065","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."},{"issue":"8","key":"10.1016\/j.nbd.2011.12.009_bb0070","doi-asserted-by":"crossref","first-page":"2163","DOI":"10.1523\/JNEUROSCI.06-08-02163.1986","article-title":"Characterization of ameboid microglia isolated from developing mammalian brain","volume":"6","author":"Giulian","year":"1986","journal-title":"J. Neurosci."},{"issue":"5369","key":"10.1016\/j.nbd.2011.12.009_bb0075","doi-asserted-by":"crossref","first-page":"1610","DOI":"10.1126\/science.280.5369.1610","article-title":"Axonal swellings and degeneration in mice lacking the major proteolipid of myelin","volume":"280","author":"Griffiths","year":"1998","journal-title":"Science"},{"issue":"52","key":"10.1016\/j.nbd.2011.12.009_bb0080","doi-asserted-by":"crossref","first-page":"39971","DOI":"10.1074\/jbc.M605095200","article-title":"Cathepsin D, a lysosomal protease, regulates ABCA1-mediated lipid efflux","volume":"281","author":"Haidar","year":"2006","journal-title":"J. Biol. Chem."},{"issue":"1","key":"10.1016\/j.nbd.2011.12.009_bb0085","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0387-7604(12)80271-3","article-title":"Development of myelination in the human fetal and infant cerebrum: a myelin basic protein immunohistochemical study","volume":"14","author":"Hasegawa","year":"1992","journal-title":"Brain Dev."},{"issue":"2","key":"10.1016\/j.nbd.2011.12.009_bb0090","doi-asserted-by":"crossref","first-page":"123","DOI":"10.1006\/mgme.2000.2961","article-title":"Expression of palmitoyl protein thioesterase in neurons","volume":"69","author":"Heinonen","year":"2000","journal-title":"Mol. Genet. Metab."},{"issue":"4","key":"10.1016\/j.nbd.2011.12.009_bb0100","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1212\/WNL.55.4.579","article-title":"Phenotype-genotype correlation in eight patients with Finnish variant late infantile NCL (CLN5)","volume":"55","author":"Holmberg","year":"2000","journal-title":"Neurology"},{"issue":"1","key":"10.1016\/j.nbd.2011.12.009_bb0095","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/j.nbd.2003.12.019","article-title":"The mouse ortholog of the neuronal ceroid lipofuscinosis CLN5 gene encodes a soluble lysosomal glycoprotein expressed in the developing brain","volume":"16","author":"Holmberg","year":"2004","journal-title":"Neurobiol. Dis."},{"issue":"1","key":"10.1016\/j.nbd.2011.12.009_bb0105","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1038\/ng0193-73","article-title":"The molecular basis of the human serum paraoxonase activity polymorphism","volume":"3","author":"Humbert","year":"1993","journal-title":"Nat. Genet."},{"issue":"8","key":"10.1016\/j.nbd.2011.12.009_bb0110","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."},{"issue":"1","key":"10.1016\/j.nbd.2011.12.009_bb0115","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0169-328X(98)00040-0","article-title":"Microglia-specific localisation of a novel calcium binding protein, Iba1","volume":"57","author":"Ito","year":"1998","journal-title":"Brain Res. Mol. Brain Res."},{"issue":"3","key":"10.1016\/j.nbd.2011.12.009_bb0120","doi-asserted-by":"crossref","first-page":"1415","DOI":"10.1111\/j.1471-4159.2008.05497.x","article-title":"Accumulation of bis(monoacylglycero)phosphate and gangliosides in mouse models of neuronal ceroid lipofuscinosis","volume":"106","author":"Jabs","year":"2008","journal-title":"J. Neurochem."},{"issue":"10","key":"10.1016\/j.nbd.2011.12.009_bb0125","doi-asserted-by":"crossref","first-page":"934","DOI":"10.1016\/j.bbadis.2006.09.001","article-title":"From genes to systems: new global strategies for the characterization of NCL biology","volume":"1762","author":"Jalanko","year":"2006","journal-title":"Biochim. Biophys. Acta"},{"issue":"2","key":"10.1016\/j.nbd.2011.12.009_bb0130","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1016\/j.ymeth.2004.11.006","article-title":"Determination of human plasma phospholipid transfer protein mass and activity","volume":"36","author":"Jauhiainen","year":"2005","journal-title":"Methods"},{"issue":"5","key":"10.1016\/j.nbd.2011.12.009_bb0135","doi-asserted-by":"crossref","first-page":"1051","DOI":"10.1046\/j.1471-4159.2003.01602.x","article-title":"Analysis of phospholipid molecular species in brains from patients with infantile and juvenile neuronal-ceroid lipofuscinosis using liquid chromatography-electrospray ionization mass spectrometry","volume":"84","author":"Kakela","year":"2003","journal-title":"J. Neurochem."},{"issue":"1","key":"10.1016\/j.nbd.2011.12.009_bb0140","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1016\/j.nbd.2006.09.001","article-title":"Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis","volume":"25","author":"Kielar","year":"2007","journal-title":"Neurobiol. Dis."},{"issue":"2","key":"10.1016\/j.nbd.2011.12.009_bb0145","doi-asserted-by":"crossref","first-page":"425","DOI":"10.1016\/S0021-9150(01)00594-9","article-title":"Dietary determinants of serum paraoxonase activity in healthy humans","volume":"160","author":"Kleemola","year":"2002","journal-title":"Atherosclerosis"},{"issue":"4","key":"10.1016\/j.nbd.2011.12.009_bb0150","doi-asserted-by":"crossref","first-page":"400","DOI":"10.1097\/00005072-195312040-00008","article-title":"A method for the combined staining of cells and fibers in the nervous system","volume":"12","author":"Kluver","year":"1953","journal-title":"J. Neuropathol. Exp. Neurol."},{"issue":"23","key":"10.1016\/j.nbd.2011.12.009_bb0155","doi-asserted-by":"crossref","first-page":"2893","DOI":"10.1093\/hmg\/ddh312","article-title":"A mouse model for Finnish variant late infantile neuronal ceroid lipofuscinosis, CLN5, reveals neuropathology associated with early aging","volume":"13","author":"Kopra","year":"2004","journal-title":"Hum. Mol. Genet."},{"issue":"1","key":"10.1016\/j.nbd.2011.12.009_bb0370","doi-asserted-by":"crossref","first-page":"42","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":"11","key":"10.1016\/j.nbd.2011.12.009_bb0165","doi-asserted-by":"crossref","first-page":"1186","DOI":"10.1038\/nm1196-1186","article-title":"Structural and functional diversity of paraoxonases","volume":"2","author":"La Du","year":"1996","journal-title":"Nat. Med."},{"issue":"3","key":"10.1016\/j.nbd.2011.12.009_bb0170","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1038\/ng1095","article-title":"Disruption of Cnp1 uncouples oligodendroglial functions in axonal support and myelination","volume":"33","author":"Lappe-Siefke","year":"2003","journal-title":"Nat. Genet."},{"issue":"3","key":"10.1016\/j.nbd.2011.12.009_bb0175","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1002\/ana.20406","article-title":"Glial fibrillary acidic protein mutations in infantile, juvenile, and adult forms of Alexander disease","volume":"57","author":"Li","year":"2005","journal-title":"Ann. Neurol."},{"issue":"6","key":"10.1016\/j.nbd.2011.12.009_bb0180","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1161\/ATVBAHA.108.179481","article-title":"Rab8 regulates ABCA1 cell surface expression and facilitates cholesterol efflux in primary human macrophages","volume":"29","author":"Linder","year":"2009","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"issue":"4","key":"10.1016\/j.nbd.2011.12.009_bb0185","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1006\/meth.2001.1262","article-title":"Analysis of relative gene expression data using real-time quantitative PCR and the 2(\u2212Delta Delta C(T)) Method","volume":"25","author":"Livak","year":"2001","journal-title":"Methods"},{"issue":"4","key":"10.1016\/j.nbd.2011.12.009_bb0190","doi-asserted-by":"crossref","first-page":"155","DOI":"10.3109\/10520297009067471","article-title":"The binding of luxol fast blue ARN by various biological lipids","volume":"45","author":"Lycette","year":"1970","journal-title":"Stain Technol."},{"issue":"10","key":"10.1016\/j.nbd.2011.12.009_bb0195","doi-asserted-by":"crossref","first-page":"1406","DOI":"10.1093\/hmg\/ddn028","article-title":"Deficiency of the INCL protein Ppt1 results in changes in ectopic F1-ATP synthase and altered cholesterol metabolism","volume":"17","author":"Lyly","year":"2008","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2011.12.009_bb0200","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1186\/1471-2121-10-83","article-title":"Novel interactions of CLN5 support molecular networking between Neuronal Ceroid Lipofuscinosis proteins","volume":"10","author":"Lyly","year":"2009","journal-title":"BMC Cell Biol."},{"issue":"6918","key":"10.1016\/j.nbd.2011.12.009_bb0205","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1038\/nature01250","article-title":"Ectopic beta-chain of ATP synthase is an apolipoprotein A-I receptor in hepatic HDL endocytosis","volume":"421","author":"Martinez","year":"2003","journal-title":"Nature"},{"issue":"1\u20133","key":"10.1016\/j.nbd.2011.12.009_bb0210","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1016\/j.bbalip.2004.08.009","article-title":"Lipid and cholesterol trafficking in NPC","volume":"1685","author":"Mukherjee","year":"2004","journal-title":"Biochim. Biophys. Acta"},{"issue":"5","key":"10.1016\/j.nbd.2011.12.009_bb0215","doi-asserted-by":"crossref","first-page":"570","DOI":"10.1002\/ana.20975","article-title":"CLN3P, the Batten's disease protein, is a novel palmitoyl-protein Delta-9 desaturase","volume":"60","author":"Narayan","year":"2006","journal-title":"Ann. Neurol."},{"issue":"52","key":"10.1016\/j.nbd.2011.12.009_bb0220","doi-asserted-by":"crossref","first-page":"52379","DOI":"10.1074\/jbc.M310695200","article-title":"Phospholipid transfer protein interacts with and stabilizes ATP-binding cassette transporter A1 and enhances cholesterol efflux from cells","volume":"278","author":"Oram","year":"2003","journal-title":"J. Biol. Chem."},{"issue":"1","key":"10.1016\/j.nbd.2011.12.009_bb0225","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1016\/j.nbd.2005.01.025","article-title":"Glial activation spreads from specific cerebral foci and precedes neurodegeneration in presymptomatic ovine neuronal ceroid lipofuscinosis (CLN6)","volume":"20","author":"Oswald","year":"2005","journal-title":"Neurobiol. Dis."},{"issue":"12","key":"10.1016\/j.nbd.2011.12.009_bb0230","doi-asserted-by":"crossref","first-page":"1339","DOI":"10.1002\/glia.20702","article-title":"Production, characterization, and efficient transfection of highly pure oligodendrocyte precursor cultures from mouse embryonic neural progenitors","volume":"56","author":"Pedraza","year":"2008","journal-title":"Glia"},{"issue":"4","key":"10.1016\/j.nbd.2011.12.009_bb0235","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1038\/nrneurol.2010.17","article-title":"Microglia in neurodegenerative disease","volume":"6","author":"Perry","year":"2010","journal-title":"Nat. Rev. Neurol."},{"issue":"2\u20133","key":"10.1016\/j.nbd.2011.12.009_bb0240","doi-asserted-by":"crossref","first-page":"314","DOI":"10.1007\/s12035-009-8096-6","article-title":"Deregulated sphingolipid metabolism and membrane organization in neurodegenerative disorders","volume":"41","author":"Piccinini","year":"2010","journal-title":"Mol. Neurobiol."},{"issue":"3","key":"10.1016\/j.nbd.2011.12.009_bb0245","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1016\/j.nbd.2005.05.018","article-title":"Thalamocortical neuron loss and localized astrocytosis in the Cln3Deltaex7\/8 knock-in mouse model of Batten disease","volume":"20","author":"Pontikis","year":"2005","journal-title":"Neurobiol. Dis."},{"issue":"6","key":"10.1016\/j.nbd.2011.12.009_bb0255","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1038\/14084","article-title":"Cholesterol modulates membrane traffic along the endocytic pathway in sphingolipid-storage diseases","volume":"1","author":"Puri","year":"1999","journal-title":"Nat. Cell Biol."},{"issue":"3","key":"10.1016\/j.nbd.2011.12.009_bb0260","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1083\/jcb.200102084","article-title":"Clathrin-dependent and -independent internalization of plasma membrane sphingolipids initiates two Golgi targeting pathways","volume":"154","author":"Puri","year":"2001","journal-title":"J. Cell Biol."},{"issue":"23","key":"10.1016\/j.nbd.2011.12.009_bb0250","doi-asserted-by":"crossref","first-page":"20961","DOI":"10.1074\/jbc.M300304200","article-title":"Sphingolipid storage induces accumulation of intracellular cholesterol by stimulating SREBP-1 cleavage","volume":"278","author":"Puri","year":"2003","journal-title":"J. Biol. Chem."},{"issue":"7","key":"10.1016\/j.nbd.2011.12.009_bb0265","doi-asserted-by":"crossref","first-page":"685","DOI":"10.1002\/(SICI)1097-0223(199907)19:7<685::AID-PD603>3.0.CO;2-F","article-title":"Prenatal diagnosis of variant late infantile neuronal ceroid lipofuscinosis (vLINCL[Finnish]; CLN5)","volume":"19","author":"Rapola","year":"1999","journal-title":"Prenat. Diagn."},{"issue":"4","key":"10.1016\/j.nbd.2011.12.009_bb0270","doi-asserted-by":"crossref","first-page":"468","DOI":"10.1038\/nn1426","article-title":"High cholesterol level is essential for myelin membrane growth","volume":"8","author":"Saher","year":"2005","journal-title":"Nat. Neurosci."},{"issue":"2","key":"10.1016\/j.nbd.2011.12.009_bb0275","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1016\/S0387-7604(88)80075-5","article-title":"Neuronal ceroid-lipofuscinoses in childhood","volume":"10","author":"Santavuori","year":"1988","journal-title":"Brain Dev."},{"issue":"3","key":"10.1016\/j.nbd.2011.12.009_bb0285","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1055\/s-2008-1059612","article-title":"A variant of Jansky-Bielschowsky disease","volume":"13","author":"Santavuori","year":"1982","journal-title":"Neuropediatrics"},{"issue":"2","key":"10.1016\/j.nbd.2011.12.009_bb0280","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1055\/s-2008-1071423","article-title":"The spectrum of Jansky-Bielschowsky disease","volume":"22","author":"Santavuori","year":"1991","journal-title":"Neuropediatrics"},{"issue":"3","key":"10.1016\/j.nbd.2011.12.009_bb0290","doi-asserted-by":"crossref","first-page":"356","DOI":"10.1002\/humu.21195","article-title":"The neuronal ceroid lipofuscinosis protein CLN5: new insights into cellular maturation, transport, and consequences of mutations","volume":"31","author":"Schmiedt","year":"2010","journal-title":"Hum. Mutat."},{"issue":"22","key":"10.1016\/j.nbd.2011.12.009_bb0295","doi-asserted-by":"crossref","first-page":"4497","DOI":"10.1093\/hmg\/ddq381","article-title":"Expression and lysosomal targeting of CLN7, a major facilitator superfamily transporter associated with variant late-infantile neuronal ceroid lipofuscinosis","volume":"19","author":"Sharifi","year":"2010","journal-title":"Hum. Mol. Genet."},{"issue":"5","key":"10.1016\/j.nbd.2011.12.009_bb0300","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1002\/(SICI)1097-4547(20000601)60:5<571::AID-JNR1>3.0.CO;2-Q","article-title":"Schwann cell remyelination is restricted to astrocyte-deficient areas after transplantation into demyelinated adult rat brain","volume":"60","author":"Shields","year":"2000","journal-title":"J. Neurosci. Res."},{"issue":"7","key":"10.1016\/j.nbd.2011.12.009_bb0305","doi-asserted-by":"crossref","first-page":"983","DOI":"10.1023\/A:1021036506299","article-title":"Altered mitochondrial function in canine ceroid-lipofuscinosis","volume":"23","author":"Siakotos","year":"1998","journal-title":"Neurochem. Res."},{"issue":"10","key":"10.1016\/j.nbd.2011.12.009_bb0310","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1016\/j.bbadis.2006.05.006","article-title":"Molecular genetics of the NCLs \u2014 status and perspectives","volume":"1762","author":"Siintola","year":"2006","journal-title":"Biochim. Biophys. Acta"},{"issue":"1","key":"10.1016\/j.nbd.2011.12.009_bb0315","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1086\/518902","article-title":"The novel neuronal ceroid lipofuscinosis gene MFSD8 encodes a putative lysosomal transporter","volume":"81","author":"Siintola","year":"2007","journal-title":"Am. J. Hum. Genet."},{"issue":"1","key":"10.1016\/j.nbd.2011.12.009_bb0375","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1016\/j.expneurol.2004.05.038","article-title":"Endogenous Nkx2.2+\/Olig2+ oligodendrocyte precursor cells fail to remyelinate the demyelinated adult rat spinal cord in the absence of astrocytes","volume":"192","author":"Talbott","year":"2005","journal-title":"Exp. Neurol."},{"issue":"4","key":"10.1016\/j.nbd.2011.12.009_bb0325","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1097\/00005072-199704000-00005","article-title":"Variant late infantile neuronal ceroid-lipofuscinosis: pathology and biochemistry","volume":"56","author":"Tyynela","year":"1997","journal-title":"J. Neuropathol. Exp. Neurol."},{"issue":"4","key":"10.1016\/j.nbd.2011.12.009_bb0330","doi-asserted-by":"crossref","first-page":"1082","DOI":"10.1161\/01.ATV.20.4.1082","article-title":"Human plasma phospholipid transfer protein increases the antiatherogenic potential of high density lipoproteins in transgenic mice","volume":"20","author":"van Haperen","year":"2000","journal-title":"Arterioscler. Thromb. Vasc. Biol."},{"issue":"7","key":"10.1016\/j.nbd.2011.12.009_bb0335","doi-asserted-by":"crossref","first-page":"2410","DOI":"10.1091\/mbc.e02-01-0031","article-title":"Neuronal ceroid lipofuscinoses are connected at molecular level: interaction of CLN5 protein with CLN2 and CLN3","volume":"13","author":"Vesa","year":"2002","journal-title":"Mol. Biol. Cell"},{"issue":"6","key":"10.1016\/j.nbd.2011.12.009_bb0340","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1016\/j.ijdevneu.2008.05.003","article-title":"A correlative light and electron microscopic study of postnatal myelination in the murine corpus callosum","volume":"26","author":"Vincze","year":"2008","journal-title":"Int. J. Dev. Neurosci."},{"key":"10.1016\/j.nbd.2011.12.009_bb0355","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"},{"issue":"2","key":"10.1016\/j.nbd.2011.12.009_bb0350","doi-asserted-by":"crossref","first-page":"308","DOI":"10.1016\/j.nbd.2009.02.001","article-title":"Progressive thalamocortical neuron loss in Cln5 deficient mice: distinct effects in Finnish variant late infantile NCL","volume":"34","author":"von Schantz","year":"2009","journal-title":"Neurobiol. Dis."},{"issue":"8","key":"10.1016\/j.nbd.2011.12.009_bb0345","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1016\/S0968-0004(02)02154-0","article-title":"TRAM, LAG1 and CLN8: members of a novel family of lipid-sensing domains?","volume":"27","author":"Winter","year":"2002","journal-title":"Trends Biochem. Sci."},{"issue":"Suppl. 1","key":"10.1016\/j.nbd.2011.12.009_bb0360","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1080\/07853890701687219","article-title":"Discrete roles of apoA-I and apoE in the biogenesis of HDL species: lessons learned from gene transfer studies in different mouse models","volume":"40","author":"Zannis","year":"2008","journal-title":"Ann. Med."}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996111003846?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996111003846?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,19]],"date-time":"2025-10-19T05:06:37Z","timestamp":1760850397000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0969996111003846"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,4]]},"references-count":72,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2012,4]]}},"alternative-id":["S0969996111003846"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2011.12.009","relation":{},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2012,4]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Cln5-deficiency in mice leads to microglial activation, defective myelination and changes in lipid metabolism","name":"articletitle","label":"Article Title"},{"value":"Neurobiology of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.nbd.2011.12.009","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2011 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}