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Cell Biol."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0370","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1126\/science.1174621","article-title":"Lipid rafts as a membrane-organizing principle","volume":"327","author":"Lingwood","year":"2010","journal-title":"Science"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0375","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1007\/s12035-009-8071-2","article-title":"Myelin proteomics: molecular anatomy of an insulating sheath","volume":"40","author":"Jahn","year":"2009","journal-title":"Mol. Neurobiol."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0380","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1017\/S1740925X06000068","article-title":"New observations on the compact myelin proteome","volume":"2","author":"Roth","year":"2006","journal-title":"Neuron Glia Biol."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0385","doi-asserted-by":"crossref","first-page":"14605","DOI":"10.1073\/pnas.0905936106","article-title":"Human myelin proteome and comparative analysis with mouse myelin","volume":"106","author":"Ishii","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0390","doi-asserted-by":"crossref","first-page":"2283","DOI":"10.1021\/pr050205c","article-title":"Profiling of myelin proteins by 2D-gel electrophoresis and multidimensional liquid chromatography coupled to MALDI TOF\u2013TOF mass spectrometry","volume":"4","author":"Vanrobaeys","year":"2005","journal-title":"J. Proteome Res."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0395","doi-asserted-by":"crossref","first-page":"16369","DOI":"10.1523\/JNEUROSCI.4016-11.2011","article-title":"Quantitative and integrative proteome analysis of peripheral nerve myelin identifies novel myelin proteins and candidate neuropathy loci","volume":"31","author":"Patzig","year":"2011","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0400","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1083\/jcb.200406092","article-title":"The raft-associated protein MAL is required for maintenance of proper axon\u2013glia interactions in the central nervous system","volume":"166","author":"Schaeren-Wiemers","year":"2004","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0405","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1083\/jcb.151.1.143","article-title":"Assembly of myelin by association of proteolipid protein with cholesterol- and galactosylceramide-rich membrane domains","volume":"151","author":"Simons","year":"2000","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0410","doi-asserted-by":"crossref","first-page":"11743","DOI":"10.1523\/JNEUROSCI.3581-06.2006","article-title":"Perturbed interactions of mutant proteolipid protein\/DM20 with cholesterol and lipid rafts in oligodendroglia: implications for dysmyelination in spastic paraplegia","volume":"26","author":"Kr\u00e4mer-Albers","year":"2006","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0415","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1002\/glia.22456","article-title":"A critical role for the cholesterol-associated proteolipids PLP and M6B in myelination of the central nervous system","volume":"61","author":"Werner","year":"2013","journal-title":"Glia"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0420","first-page":"145","article-title":"Oligodendrogenesis in the normal and pathological central nervous system","volume":"8","author":"El","year":"2014","journal-title":"Front. Neurosci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0425","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1146\/annurev-cellbio-100913-013101","article-title":"Myelination of the nervous system: mechanisms and functions","volume":"30","author":"Nave","year":"2014","journal-title":"Annu. Rev. Cell Dev. Biol."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0430","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1016\/j.neuron.2013.01.006","article-title":"Oligodendrocyte dynamics in the healthy adult CNS: evidence for myelin remodeling","volume":"77","author":"Young","year":"2013","journal-title":"Neuron"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0435","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1126\/science.1254960","article-title":"Motor skill learning requires active central myelination","volume":"346","author":"McKenzie","year":"2014","journal-title":"Science"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0440","doi-asserted-by":"crossref","first-page":"8937","DOI":"10.1074\/jbc.272.14.8937","article-title":"Oligodendrocytes direct glycosyl phosphatidylinositol-anchored proteins to the myelin sheath in glycosphingolipid-rich complexes","volume":"272","author":"Kr\u00e4mer","year":"1997","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0445","doi-asserted-by":"crossref","first-page":"499","DOI":"10.1002\/glia.20406","article-title":"Rafts in oligodendrocytes: evidence and structure\u2013function relationship","volume":"54","author":"Gielen","year":"2006","journal-title":"Glia"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0450","doi-asserted-by":"crossref","first-page":"1760","DOI":"10.1002\/jnr.22020","article-title":"Comprehensive analysis of expression, subcellular localization, and cognate pairing of SNARE proteins in oligodendrocytes","volume":"87","author":"Feldmann","year":"2009","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0455","doi-asserted-by":"crossref","first-page":"5659","DOI":"10.1523\/JNEUROSCI.6638-10.2011","article-title":"Transport of the major myelin proteolipid protein is directed by VAMP3 and VAMP7","volume":"31","author":"Feldmann","year":"2011","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0460","doi-asserted-by":"crossref","first-page":"834","DOI":"10.1242\/jcs.022731","article-title":"Distinct endocytic recycling of myelin proteins promotes oligodendroglial membrane remodeling","volume":"121","author":"Winterstein","year":"2008","journal-title":"J. Cell Sci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0465","doi-asserted-by":"crossref","first-page":"656","DOI":"10.1002\/jemt.1050","article-title":"Membrane traffic in myelinating oligodendrocytes","volume":"52","author":"Kr\u00e4mer","year":"2001","journal-title":"Microsc. Res. Tech."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0470","doi-asserted-by":"crossref","first-page":"937","DOI":"10.1083\/jcb.200509022","article-title":"Neuron to glia signaling triggers myelin membrane exocytosis from endosomal storage sites","volume":"172","author":"Trajkovic","year":"2006","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0475","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1074\/jbc.M110.190009","article-title":"Inhibition of myelin membrane sheath formation by oligodendrocyte-derived exosome-like vesicles","volume":"286","author":"Bakhti","year":"2011","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0480","doi-asserted-by":"crossref","first-page":"e1003462","DOI":"10.1371\/journal.pgen.1003462","article-title":"Npc1 acting in neurons and glia is essential for the formation and maintenance of CNS myelin","volume":"9","author":"Yu","year":"2013","journal-title":"PLoS Genet."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0485","doi-asserted-by":"crossref","first-page":"3560","DOI":"10.1523\/JNEUROSCI.4926-06.2007","article-title":"Rho regulates membrane transport in the endocytic pathway to control plasma membrane specialization in oligodendroglial cells","volume":"27","author":"Kippert","year":"2007","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0490","doi-asserted-by":"crossref","first-page":"1647","DOI":"10.1126\/science.1206998","article-title":"Control of local protein synthesis and initial events in myelination by action potentials","volume":"333","author":"Wake","year":"2011","journal-title":"Science"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0495","doi-asserted-by":"crossref","first-page":"29042","DOI":"10.1074\/jbc.274.41.29042","article-title":"Compartmentation of Fyn kinase with glycosylphosphatidylinositol-anchored molecules in oligodendrocytes facilitates kinase activation during myelination","volume":"274","author":"Kr\u00e4mer","year":"1999","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0500","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1523\/JNEUROSCI.22-03-00698.2002","article-title":"Process outgrowth of oligodendrocytes is promoted by interaction of fyn kinase with the cytoskeletal protein tau","volume":"22","author":"Klein","year":"2002","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0505","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1111\/j.1471-4159.2008.05600.x","article-title":"Truncated Tau with the Fyn-binding domain and without the microtubule-binding domain hinders the myelinating capacity of an oligodendrocyte cell line","volume":"107","author":"Belkadi","year":"2008","journal-title":"J. Neurochem."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0510","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1074\/jbc.M405973200","article-title":"Developmental abnormalities of myelin basic protein expression in fyn knock-out brain reveal a role of Fyn in posttranscriptional regulation","volume":"280","author":"Lu","year":"2005","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0515","doi-asserted-by":"crossref","first-page":"579","DOI":"10.1083\/jcb.200706164","article-title":"Activation of oligodendroglial Fyn kinase enhances translation of mRNAs transported in hnRNP A2-dependent RNA granules","volume":"181","author":"White","year":"2008","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0520","doi-asserted-by":"crossref","first-page":"1742","DOI":"10.1074\/jbc.M111.235010","article-title":"Heterogeneous nuclear ribonucleoprotein (hnRNP) F is a novel component of oligodendroglial RNA transport granules contributing to regulation of myelin basic protein (MBP) synthesis","volume":"287","author":"White","year":"2012","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0525","doi-asserted-by":"crossref","first-page":"5037","DOI":"10.1038\/sj.emboj.7601376","article-title":"Myelin basic protein-dependent plasma membrane reorganization in the formation of myelin","volume":"25","author":"Fitzner","year":"2006","journal-title":"EMBO J."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0530","doi-asserted-by":"crossref","first-page":"e1001577","DOI":"10.1371\/journal.pbio.1001577","article-title":"Myelin membrane assembly is driven by a phase transition of myelin basic proteins into a cohesive protein meshwork","volume":"11","author":"Aggarwal","year":"2013","journal-title":"PLoS Biol."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0535","doi-asserted-by":"crossref","first-page":"4794","DOI":"10.1523\/JNEUROSCI.3955-08.2009","article-title":"Phosphatidylinositol 4,5-bisphosphate-dependent interaction of myelin basic protein with the plasma membrane in oligodendroglial cells and its rapid perturbation by elevated calcium","volume":"29","author":"Nawaz","year":"2009","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0540","doi-asserted-by":"crossref","first-page":"210","DOI":"10.1016\/S0022-3476(64)80264-X","article-title":"A newly recognized syndrome of multiple congenital anomalies","author":"Smith","year":"1964","journal-title":"J. Pediatr."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0545","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1002\/ajmg.c.31346","article-title":"Mutational spectrum of Smith\u2013Lemli\u2013Opitz syndrome","volume":"160C","author":"Waterham","year":"2012","journal-title":"Am. J. Med. Genet. C: Semin. Med. Genet."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0550","article-title":"Determination of the allelic frequency in Smith\u2013Lemli\u2013Opitz syndrome by analysis of massively parallel sequencing data sets","author":"Cross","year":"2014","journal-title":"Clin. Genet."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0555","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1111\/j.1399-0004.2005.00515.x","article-title":"Recent insights into the Smith\u2013Lemli\u2013Opitz syndrome","volume":"68","author":"Yu","year":"2005","journal-title":"Clin. Genet."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0560","doi-asserted-by":"crossref","first-page":"558","DOI":"10.1136\/jmg.35.7.558","article-title":"Smith\u2013Lemli\u2013Opitz syndrome: a variable clinical and biochemical phenotype","volume":"35","author":"Ryan","year":"1998","journal-title":"J. Med. Genet."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0565","doi-asserted-by":"crossref","first-page":"1414","DOI":"10.1016\/0140-6736(93)90983-N","article-title":"Defective cholesterol biosynthesis in Smith\u2013Lemli\u2013Opitz syndrome","volume":"341","author":"Irons","year":"1993","journal-title":"Lancet"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0570","doi-asserted-by":"crossref","first-page":"905","DOI":"10.1172\/JCI200112103","article-title":"7-Dehydrocholesterol-dependent proteolysis of HMG-CoA reductase suppresses sterol biosynthesis in a mouse model of Smith\u2013Lemli\u2013Opitz\/RSH syndrome","volume":"108","author":"Fitzky","year":"2001","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0575","doi-asserted-by":"crossref","first-page":"555","DOI":"10.1093\/hmg\/10.6.555","article-title":"Biochemical, phenotypic and neurophysiological characterization of a genetic mouse model of RSH\/Smith\u2013Lemli\u2013Opitz syndrome","volume":"10","author":"Wassif","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0580","doi-asserted-by":"crossref","first-page":"802","DOI":"10.1016\/j.steroids.2007.07.002","article-title":"Cholesterol biosynthesis from birth to adulthood in a mouse model for 7-dehydrosterol reductase deficiency (Smith\u2013Lemli\u2013Opitz syndrome)","volume":"72","author":"Marcos","year":"2007","journal-title":"Steroids"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0585","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1093\/hmg\/ddl003","article-title":"Development and characterization of a hypomorphic Smith\u2013Lemli\u2013Opitz syndrome mouse model and efficacy of simvastatin therapy","volume":"15","author":"Correa-Cerro","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0590","doi-asserted-by":"crossref","first-page":"2356","DOI":"10.1172\/JCI28988","article-title":"Severe facial clefting in Insig-deficient mouse embryos caused by sterol accumulation and reversed by lovastatin","volume":"116","author":"Engelking","year":"2006","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0595","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1136\/jmg.2004.018085","article-title":"Maternal apo E genotype is a modifier of the Smith\u2013Lemli\u2013Opitz syndrome","volume":"41","author":"Witsch-Baumgartner","year":"2004","journal-title":"J. Med. Genet."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0600","doi-asserted-by":"crossref","first-page":"1876","DOI":"10.1242\/jcs.02906","article-title":"Abnormal sterols in cholesterol-deficiency diseases cause secretory granule malformation and decreased membrane curvature","volume":"119","author":"Gondre-Lewis","year":"2006","journal-title":"J. Cell Sci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0605","doi-asserted-by":"crossref","first-page":"800","DOI":"10.1016\/j.bbrc.2007.09.040","article-title":"Differential effects of cholesterol and 7-dehydrocholesterol on ligand binding of solubilized hippocampal serotonin1A receptors: implications in SLOS","volume":"363","author":"Chattopadhyay","year":"2007","journal-title":"Biochem. Biophys. Res. 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Lipid Res."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0635","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1016\/j.ymgme.2014.03.009","article-title":"Involvement and alteration of the Sonic Hedgehog pathway is associated with decreased cholesterol level in trisomy 18 and SLO amniocytes","volume":"112","author":"Gruchy","year":"2014","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0640","doi-asserted-by":"crossref","first-page":"508","DOI":"10.1038\/ng1134","article-title":"A defective response to Hedgehog signaling in disorders of cholesterol biosynthesis","volume":"33","author":"Cooper","year":"2003","journal-title":"Nat. Genet."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0645","doi-asserted-by":"crossref","first-page":"279","DOI":"10.1016\/j.mod.2009.01.007","article-title":"Trafficking, development and hedgehog","volume":"126","author":"Simpson","year":"2009","journal-title":"Mech. Dev."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0650","doi-asserted-by":"crossref","first-page":"772","DOI":"10.1038\/nrn1990","article-title":"Morphogen to mitogen: the multiple roles of hedgehog signalling in vertebrate neural development","volume":"7","author":"Fuccillo","year":"2006","journal-title":"Nat. Rev. Neurosci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0655","doi-asserted-by":"crossref","first-page":"1880","DOI":"10.2353\/ajpath.2009.080947","article-title":"Statin therapy inhibits remyelination in the central nervous system","volume":"174","author":"Miron","year":"2009","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0660","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1002\/(SICI)1096-8628(19970131)68:3<305::AID-AJMG11>3.0.CO;2-X","article-title":"Clinical effects of cholesterol supplementation in six patients with the Smith\u2013Lemli\u2013Opitz syndrome (SLOS)","volume":"68","author":"Elias","year":"1997","journal-title":"Am. J. Med. Genet."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0665","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1002\/(SICI)1096-8628(19970131)68:3<315::AID-AJMG13>3.0.CO;2-W","article-title":"Cholesterol and bile acid replacement therapy in children and adults with Smith\u2013Lemli\u2013Opitz (SLO\/RSH) syndrome","volume":"68","author":"Nwokoro","year":"1997","journal-title":"Am. J. Med. Genet."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0670","first-page":"783","article-title":"Cholesterol supplementation does not improve developmental progress in Smith\u2013Lemli\u2013Opitz syndrome","volume":"144","author":"Sikora","year":"2004","journal-title":"J. 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Lipid Res."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0685","doi-asserted-by":"crossref","first-page":"375","DOI":"10.1007\/s10545-007-0537-7","article-title":"Effects of cholesterol and simvastatin treatment in patients with Smith\u2013Lemli\u2013Opitz syndrome (SLOS)","volume":"30","author":"Haas","year":"2007","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0690","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1203\/PDR.0b013e31819ea4eb","article-title":"Effects of dietary cholesterol and simvastatin on cholesterol synthesis in Smith\u2013Lemli\u2013Opitz syndrome","volume":"65","author":"Chan","year":"2009","journal-title":"Pediatr. Res."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0695","series-title":"Ein Unbekanntes Krankheitsbild","first-page":"1","author":"Niemann","year":"1914"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0700","series-title":"Der Morbus Gaucher Und Die Ihm \u00c4hnlichen Krankheiten (Die Lipoidzellige Splenohepatomegalie Typus Niemann Und Die Diabetische Lipoidzellenhypoplasie Der Milz)","first-page":"519","author":"Pick","year":"1926"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0705","doi-asserted-by":"crossref","first-page":"1247","DOI":"10.1038\/nm.1876","article-title":"Niemann\u2013Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium","volume":"14","author":"Lloyd-Evans","year":"2008","journal-title":"Nat. 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Lipidol."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0740","doi-asserted-by":"crossref","first-page":"3107","DOI":"10.1093\/hmg\/11.24.3107","article-title":"Rescue of neurodegeneration in Niemann\u2013Pick C mice by a prion-promoter-driven Npc1 cDNA transgene","volume":"11","author":"Loftus","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0745","doi-asserted-by":"crossref","first-page":"4367","DOI":"10.1523\/JNEUROSCI.5981-10.2011","article-title":"Anatomically defined neuron-based rescue of neurodegenerative Niemann\u2013Pick type C disorder","volume":"31","author":"Lopez","year":"2011","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0750","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1212\/WNL.43.1_Part_1.61","article-title":"The effect of cholesterol-lowering agents on hepatic and plasma cholesterol in Niemann\u2013Pick disease type C","volume":"43","author":"Patterson","year":"1993","journal-title":"Neurology"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0755","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1038\/sj.bmt.1701826","article-title":"Niemann\u2013Pick disease type C (a cellular cholesterol lipidosis) treated by bone marrow transplantation","volume":"24","author":"Hsu","year":"1999","journal-title":"Bone Marrow Transplant."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0760","doi-asserted-by":"crossref","first-page":"1710","DOI":"10.1194\/jlr.M700125-JLR200","article-title":"Receptor-mediated and bulk-phase endocytosis cause macrophage and cholesterol accumulation in Niemann\u2013Pick C disease","volume":"48","author":"Liu","year":"2007","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0765","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1007\/BF00599187","article-title":"Hypoplasia of the corpus callosum in Niemann\u2013Pick type C disease","volume":"36","author":"Palmeri","year":"1994","journal-title":"Neuroradiology"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0770","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1016\/0005-2760(80)90116-2","article-title":"A lysosomal storage disorder in mice characterized by a dual deficiency of sphingomyelinase and glucocerebrosidase","volume":"619","author":"Pentchev","year":"1980","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0775","doi-asserted-by":"crossref","first-page":"374","DOI":"10.1007\/BF00687215","article-title":"Dysmyelination in NCTR-Balb\/C mouse mutant with a lysosomal storage disorder. 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Biol. Chem."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0805","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1016\/S1388-1981(99)00070-0","article-title":"A porcine homolog of the major secretory protein of human epididymis, HE1, specifically binds cholesterol","volume":"1438","author":"Okamura","year":"1999","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0810","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1006\/mgme.2000.3061","article-title":"The structure and function of the Niemann\u2013Pick C1 protein","volume":"71","author":"Ioannou","year":"2000","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0815","doi-asserted-by":"crossref","first-page":"657","DOI":"10.1038\/35089558","article-title":"Multidrug permeases and subcellular cholesterol transport","volume":"2","author":"Ioannou","year":"2001","journal-title":"Nat. Rev. Mol. 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Biochem."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0845","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/j.jneumeth.2007.09.002","article-title":"Partial blockage of sterol biosynthesis with a squalene synthase inhibitor in early postnatal Niemann\u2013Pick type C npcnih null mice brains reduces neuronal cholesterol accumulation, abrogates astrogliosis, but may inhibit myelin maturation","volume":"168","author":"Reid","year":"2008","journal-title":"J. Neurosci. Methods"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0850","doi-asserted-by":"crossref","first-page":"311","DOI":"10.1111\/j.1471-4159.2010.06768.x","article-title":"Pre-synaptic defects of NPC1-deficient hippocampal neurons are not directly related to plasma membrane cholesterol","volume":"114","author":"Hawes","year":"2010","journal-title":"J. Neurochem."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0855","doi-asserted-by":"crossref","first-page":"1743","DOI":"10.1021\/cr970022c","article-title":"Introduction and general overview of cyclodextrin chemistry","volume":"98","author":"Szejtli","year":"1998","journal-title":"Chem. Rev."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0860","doi-asserted-by":"crossref","first-page":"16026","DOI":"10.1074\/jbc.271.27.16026","article-title":"Cellular cholesterol efflux mediated by cyclodextrins. Demonstration of kinetic pools and mechanism of efflux","volume":"271","author":"Yancey","year":"1996","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0865","doi-asserted-by":"crossref","first-page":"1311","DOI":"10.1016\/j.bbamem.2007.03.026","article-title":"Use of cyclodextrins to manipulate plasma membrane cholesterol content: evidence, misconceptions and control strategies","volume":"1768","author":"Zidovetzki","year":"2007","journal-title":"Biochim. Biophys. 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Res."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0880","doi-asserted-by":"crossref","first-page":"e6951","DOI":"10.1371\/journal.pone.0006951","article-title":"Chronic cyclodextrin treatment of murine Niemann\u2013Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression","volume":"4","author":"Davidson","year":"2009","journal-title":"PLoS One"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0885","doi-asserted-by":"crossref","first-page":"2802","DOI":"10.1002\/jnr.21707","article-title":"MRI detects therapeutic effects in weanling Niemann\u2013Pick type C mice","volume":"86","author":"Lope-Piedrafita","year":"2008","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0890","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1016\/S0024-3205(01)01384-4","article-title":"Cyclodextrins in the treatment of a mouse model of Niemann\u2013Pick C disease","volume":"70","author":"Camargo","year":"2001","journal-title":"Life Sci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0895","doi-asserted-by":"crossref","first-page":"491","DOI":"10.1007\/s10545-012-9583-x","article-title":"Cyclodextrin alleviates neuronal storage of cholesterol in Niemann\u2013Pick C disease without evidence of detectable blood\u2013brain barrier permeability","volume":"36","author":"Pontikis","year":"2013","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0900","doi-asserted-by":"crossref","first-page":"9404","DOI":"10.1523\/JNEUROSCI.1317-11.2011","article-title":"Unesterified cholesterol accumulation in late endosomes\/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment","volume":"31","author":"Aqul","year":"2011","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0905","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/j.ymgme.2012.11.005","article-title":"Effects of cyclodextrin in two patients with Niemann\u2013Pick type C disease","volume":"108","author":"Matsuo","year":"2013","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0910","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1007\/BF02057569","article-title":"\u00dcber Eine Eigent\u00fcmliche Form Spastischer L\u00e4hmung Mit Cerebralerscheinungen Auf Hereditarer Grundlage (Multiple Sklerose)","author":"Pelizaeus","year":"1885","journal-title":"Arch. Psychiat. Nervenkr."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0915","series-title":"Gesetzmaessigkeiten in Der Vererbung Und Verbreitung Verschiedener Hereditaer-Familiaerer Erkrankungen","first-page":"172","author":"Merzbacher","year":"1909"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0920","doi-asserted-by":"crossref","first-page":"4447","DOI":"10.1073\/pnas.92.10.4447","article-title":"Dominant-negative action of the jimpy mutation in mice complemented with an autosomal transgene for myelin proteolipid protein","volume":"92","author":"Schneider","year":"1995","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0925","doi-asserted-by":"crossref","first-page":"155ra137","DOI":"10.1126\/scitranslmed.3004373","article-title":"Neural stem cell engraftment and myelination in the human brain","volume":"4","author":"Gupta","year":"2012","journal-title":"Sci. Transl. Med."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0930","doi-asserted-by":"crossref","first-page":"1723","DOI":"10.1002\/jnr.23458","article-title":"Diffusion tensor imaging of patients with proteolipid protein 1 gene mutations","volume":"92","author":"Laukka","year":"2014","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0935","doi-asserted-by":"crossref","first-page":"4455","DOI":"10.1021\/bi00852a042","article-title":"The maturation of human white matter myelin. 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Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0955","doi-asserted-by":"crossref","first-page":"1873","DOI":"10.1111\/j.1471-4159.1987.tb02449.x","article-title":"A single nucleotide difference in the gene for myelin proteolipid protein defines the jimpy mutation in mouse","volume":"49","author":"Nave","year":"1987","journal-title":"J. Neurochem."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0960","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1523\/JNEUROSCI.10-01-00117.1990","article-title":"Conservative amino acid substitution in the myelin proteolipid protein of jimpymsd mice","volume":"10","author":"Gencic","year":"1990","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0965","first-page":"322","article-title":"Jimpy, a new totally sexlinked gene in the house mouse","volume":"86","author":"Phillips","year":"1954","journal-title":"Z. Indukt. Abstamm. Vererbungsl."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0970","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1126\/science.144.3616.309","article-title":"Mutant mice (Quaking and Jimpy) with deficient myelination in the central nervous system","volume":"144","author":"Sidman","year":"1964","journal-title":"Science"},{"key":"10.1016\/j.bbalip.2015.02.010_bb0975","doi-asserted-by":"crossref","first-page":"2813","DOI":"10.1523\/JNEUROSCI.06-10-02813.1986","article-title":"Oligodendroglial cell death in jimpy mice: an explanation for the myelin deficit","volume":"6","author":"Knapp","year":"1986","journal-title":"J. Neurosci."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0980","doi-asserted-by":"crossref","first-page":"574","DOI":"10.1002\/jnr.490370504","article-title":"Many naturally occurring mutations of myelin proteolipid protein impair its intracellular transport","volume":"37","author":"Gow","year":"1994","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0985","doi-asserted-by":"crossref","first-page":"422","DOI":"10.1038\/ng0896-422","article-title":"A cellular mechanism governing the severity of Pelizaeus\u2013Merzbacher disease","volume":"13","author":"Gow","year":"1996","journal-title":"Nat. Genet."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0990","doi-asserted-by":"crossref","first-page":"925","DOI":"10.1083\/jcb.140.4.925","article-title":"Disrupted proteolipid protein trafficking results in oligodendrocyte apoptosis in an animal model of Pelizaeus\u2013Merzbacher disease","volume":"140","author":"Gow","year":"1998","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.bbalip.2015.02.010_bb0995","doi-asserted-by":"crossref","first-page":"4342","DOI":"10.1073\/pnas.0407287102","article-title":"Disease-associated mutations cause premature oligomerization of myelin proteolipid protein in the endoplasmic reticulum","volume":"102","author":"Swanton","year":"2005","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.bbalip.2015.02.010_bb1000","doi-asserted-by":"crossref","first-page":"17813","DOI":"10.1073\/pnas.0704975104","article-title":"A common mechanism of PLP\/DM20 misfolding causes cysteine-mediated endoplasmic reticulum retention in oligodendrocytes and Pelizaeus\u2013Merzbacher disease","volume":"104","author":"Dhaunchak","year":"2007","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.bbalip.2015.02.010_bb1005","doi-asserted-by":"crossref","first-page":"273","DOI":"10.1007\/BF01217305","article-title":"Rumpshaker mouse: a new X-linked mutation affecting myelination: evidence for a defect in PLP expression","volume":"19","author":"Griffiths","year":"1990","journal-title":"J. Neurocytol."},{"key":"10.1016\/j.bbalip.2015.02.010_bb1010","doi-asserted-by":"crossref","first-page":"758","DOI":"10.1038\/358758a0","article-title":"Uncoupling of hypomyelination and glial cell death by a mutation in the proteolipid protein gene","volume":"358","author":"Schneider","year":"1992","journal-title":"Nature"},{"key":"10.1016\/j.bbalip.2015.02.010_bb1015","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1007\/s00401-003-0808-9","article-title":"Age-related axonal and myelin changes in the rumpshaker mutation of the Plp gene","volume":"107","author":"Edgar","year":"2004","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.bbalip.2015.02.010_bb1030","doi-asserted-by":"crossref","first-page":"867","DOI":"10.1016\/0014-4886(83)90050-X","article-title":"Cholesterol ester content and activities of the ester metabolizing enzymes in jpmsd mouse brain","volume":"79","author":"Johnson","year":"1983","journal-title":"Exp. 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