{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T13:31:15Z","timestamp":1777555875167,"version":"3.51.4"},"reference-count":46,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2018,2,1]],"date-time":"2018-02-01T00:00:00Z","timestamp":1517443200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Gene"],"published-print":{"date-parts":[[2018,2]]},"DOI":"10.1016\/j.gene.2017.12.006","type":"journal-article","created":{"date-parts":[[2017,12,7]],"date-time":"2017-12-07T14:38:48Z","timestamp":1512657528000},"page":"117-123","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":18,"special_numbering":"C","title":["In Niemann-Pick C1 mouse models, glial-only expression of the normal gene extends survival much further than do changes in genetic background or treatment with hydroxypropyl-beta-cyclodextrin"],"prefix":"10.1016","volume":"643","author":[{"given":"Craig A.","family":"Marshall","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dawn E.","family":"Watkins-Chow","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giampiero","family":"Palladino","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gail","family":"Deutsch","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keshav","family":"Chandran","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"William J.","family":"Pavan","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert P.","family":"Erickson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.gene.2017.12.006_bb0005","series-title":"Inbred and Genetically Defined Strains of Laboratory Animals, III, Part I, Mouse and Rat","first-page":"45","author":"Altman","year":"1979"},{"key":"10.1016\/j.gene.2017.12.006_bb0010","doi-asserted-by":"crossref","first-page":"886","DOI":"10.1002\/hep.20868","article-title":"Cholesterol accumulation and liver cell death in mice with Niemann-Pick type C disease","volume":"42","author":"Beltroy","year":"2005","journal-title":"Hepatology"},{"key":"10.1016\/j.gene.2017.12.006_bb0015","doi-asserted-by":"crossref","first-page":"1319","DOI":"10.1038\/ng1479","article-title":"Polymorphisms in FKBP5 are associated with increased recurrence of depressive episodes and rapid response to antidepressant treatment","volume":"36","author":"Binder","year":"2004","journal-title":"Nat. Genet."},{"key":"10.1016\/j.gene.2017.12.006_bb0020","doi-asserted-by":"crossref","first-page":"875","DOI":"10.3233\/JAD-2012-120199","article-title":"Niemann-Pick C1 mice, a model of \u201cjuvenile Alzheimer's disease\u201d, with normal gene expression in neurons and fibrillary astrocytes show long term survival and delayed neurodegeneration","volume":"30","author":"Borbon","year":"2012","journal-title":"J. Alzheimers Dis."},{"key":"10.1016\/j.gene.2017.12.006_bb0025","doi-asserted-by":"crossref","first-page":"1030","DOI":"10.1523\/JNEUROSCI.14-03-01030.1994","article-title":"GFAP promoter directs astrocyte-specific expression in transgenic mice","volume":"14","author":"Brenner","year":"1994","journal-title":"J. Neurosci."},{"key":"10.1016\/j.gene.2017.12.006_bb0030","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1038\/nrn2652","article-title":"Tests to assess motor phenotype in mice: a user's guide","volume":"10","author":"Brooks","year":"2009","journal-title":"Nat. Rev. Neurosci."},{"key":"10.1016\/j.gene.2017.12.006_bb0035","doi-asserted-by":"crossref","first-page":"65","DOI":"10.1016\/j.mcn.2014.06.003","article-title":"Relevance of neuronal and glial NPC1 for synaptic input to cerebellar Purkinje cells","volume":"61","author":"Buard","year":"2014","journal-title":"Mol. Cell. Neurosci."},{"key":"10.1016\/j.gene.2017.12.006_bb0040","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-Pick C disease","volume":"70","author":"Camargo","year":"2001","journal-title":"Life Sci."},{"key":"10.1016\/j.gene.2017.12.006_bb0045","doi-asserted-by":"crossref","first-page":"2277","DOI":"10.1093\/hmg\/ddx118","article-title":"Shortened primary cilium length and dysregulated sonic hedgehog signaling in Niemann-Pick C1 disease","volume":"26","author":"Canterini","year":"2017","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.gene.2017.12.006_bb0050","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1186\/s40478-016-0370-z","article-title":"A delay in the acquisition of complex motor skills in murine models of NPC disease underscores neuronal and glial cell defects of postnatally developing cerebellum","volume":"4","author":"Caporali","year":"2016","journal-title":"Acta Neuropathol. Commun."},{"key":"10.1016\/j.gene.2017.12.006_bb0055","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1016\/j.mcn.2009.10.007","article-title":"Lack of Niemann-Pick type C1 induces age-related degeneration in the mouse retina","volume":"43","author":"Claudepierre","year":"2010","journal-title":"Mol. Cell. Neurosci."},{"issue":"9","key":"10.1016\/j.gene.2017.12.006_bb1000","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.gene.2017.12.006_bb0060","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1016\/j.gene.2015.12.033","article-title":"Extensive macrophage accumulation in young and old Niemann-Pick C1 model mice involves the alternative, M2, activation pathway and inhibition of macrophage apoptosis","volume":"578","author":"Deutsch","year":"2016","journal-title":"Gene"},{"key":"10.1016\/j.gene.2017.12.006_bb0065","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1007\/BF03195698","article-title":"Expression of Npc1 in glial cells corrects sterility in Npc1\u2212\/\u2212 mice","volume":"50","author":"Donohue","year":"2009","journal-title":"J. Appl. Genet."},{"key":"10.1016\/j.gene.2017.12.006_bb0070","doi-asserted-by":"crossref","first-page":"837","DOI":"10.1093\/hmg\/ddp552","article-title":"Conditional Niemann\u2013Pick C mice demonstrate cell autonomous Purkinje cell degeneration","volume":"19","author":"Elrick","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.gene.2017.12.006_bb0075","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1007\/BF00486617","article-title":"Erythrocytic nicotinamide-adenine dinucleotide phosphate levels and the genetic regulation of erythrocytic glucose-6-phosphate dehydrogenase activity in the inbred mouse","volume":"11","author":"Erickson","year":"1974","journal-title":"Biochem. Genet."},{"key":"10.1016\/j.gene.2017.12.006_bb0080","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1007\/s13353-012-0130-0","article-title":"Current controversies in Niemann-Pick C1 disease: steroids or gangliosides; neurons or neurons and glia","volume":"54","author":"Erickson","year":"2013","journal-title":"J. Appl. Genet."},{"key":"10.1016\/j.gene.2017.12.006_bb0085","doi-asserted-by":"crossref","first-page":"G300","DOI":"10.1152\/ajpgi.00568.2004","article-title":"Liver disease with altered bile acid transport in Niemann-Pick C mice on a high fat, 1% cholesterol diet","volume":"289","author":"Erickson","year":"2005","journal-title":"Am. J. Physiol. Liver GI"},{"key":"10.1016\/j.gene.2017.12.006_bb0090","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1002\/jcb.21200","article-title":"Characterization of liver disease and lipid metabolism in the Niemann-Pick C1 mouse","volume":"101","author":"Garver","year":"2007","journal-title":"J. Cell. Biochem."},{"key":"10.1016\/j.gene.2017.12.006_bb0095","doi-asserted-by":"crossref","first-page":"704","DOI":"10.1038\/nm1073","article-title":"Niemann\u2013Pick type C disease involves disrupted neurosteroidogenesis and responds to allopregnanolone","volume":"10","author":"Griffin","year":"2004","journal-title":"Nat. Med."},{"key":"10.1016\/j.gene.2017.12.006_bb0100","first-page":"5","article-title":"Niemann-pick C disease: recent advances","volume":"3","author":"Heidenreich","year":"1997","journal-title":"J. Rare Dis."},{"key":"10.1016\/j.gene.2017.12.006_bb0105","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0082216","article-title":"Olfactory deficits in Niemann-Pick C1 (NPC1) disease","volume":"8","author":"Hovakimyan","year":"2013","journal-title":"PLoS ONE"},{"key":"10.1016\/j.gene.2017.12.006_bb0110","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/BF00487005","article-title":"The influence of genetic background on expression of mutations at the diabetes locus in the mouse. IC57BL-KsJ and C57BL-6J strains","volume":"7","author":"Hummel","year":"1972","journal-title":"Biochem. Genet."},{"key":"10.1016\/j.gene.2017.12.006_bb0115","doi-asserted-by":"crossref","first-page":"1457","DOI":"10.1038\/oby.2009.415","article-title":"Decreased Npc1 gene dosage in mice is associated with weight gain","volume":"18","author":"Jelinek","year":"2010","journal-title":"Obesity"},{"key":"10.1016\/j.gene.2017.12.006_bb0120","doi-asserted-by":"crossref","first-page":"289","DOI":"10.1038\/nature10413","article-title":"Mouse genomic variation and its effect on phenotypes and gene regulation","volume":"477","author":"Keane","year":"2011","journal-title":"Nature"},{"key":"10.1016\/j.gene.2017.12.006_bb0125","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1007\/s10162-014-0459-7","article-title":"Hearing loss is an early consequence of Npc1 gene deletion in themouse model of Niemann-Pick disease, type C","volume":"15","author":"King","year":"2014","journal-title":"J. Assoc. Res. Otolaryngol."},{"key":"10.1016\/j.gene.2017.12.006_bb0130","doi-asserted-by":"crossref","first-page":"1627","DOI":"10.1074\/jbc.M610001200","article-title":"Lipid homeostasis and lipoprotein secretion in Niemann-Pick C1-deficient hepatocytes","volume":"282","author":"Kulinski","year":"2007","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.gene.2017.12.006_bb0135","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1194\/jlr.M700525-JLR200","article-title":"Genetic variations and treatments that affect the lifespan of NPC1 mice","volume":"49","author":"Liu","year":"2008","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.gene.2017.12.006_bb0140","doi-asserted-by":"crossref","first-page":"2377","DOI":"10.1073\/pnas.0810895106","article-title":"Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the Npc1\u2212\/\u2212 mouse","volume":"106","author":"Liu","year":"2009","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.gene.2017.12.006_bb0145","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1126\/science.277.5323.232","article-title":"Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene","volume":"277","author":"Loftus","year":"1997","journal-title":"Science"},{"key":"10.1016\/j.gene.2017.12.006_bb0150","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1093\/hmg\/ddr505","article-title":"A novel mouse model of Niemann-Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations","volume":"21","author":"Maue","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.gene.2017.12.006_bb0155","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1038\/ng.301","article-title":"Genome-wide association study for early-onset and morbid adult obesity identifies three new risk loci in European populations","volume":"41","author":"Meyre","year":"2009","journal-title":"Nat. Genet."},{"key":"10.1016\/j.gene.2017.12.006_bb0160","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1093\/oxfordjournals.jhered.a109635","article-title":"Sphingomyelinosis, a new mutation in the mouse. A model of Niemann-Pick disease in humans","volume":"73","author":"Miyawaki","year":"1982","journal-title":"J. Hered."},{"key":"10.1016\/j.gene.2017.12.006_bb0165","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1093\/oxfordjournals.jhered.a110265","article-title":"A mouse model for Niemann-Pick disease. Influence of genetic background on disease expression in spm\/spm mice","volume":"77","author":"Miyawaki","year":"1986","journal-title":"J. Hered."},{"key":"10.1016\/j.gene.2017.12.006_bb0170","first-page":"140","article-title":"Lysosome lipid storage in NCTR-BALB\/c mice. I. Description of the disease and genetics","volume":"108","author":"Morris","year":"1982","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.gene.2017.12.006_bb0175","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1016\/j.nbd.2014.06.012","article-title":"A marked paucity of granule cells in the developing cerebellum of the Npc1\u2212\/\u2212 mouse is corrected by a single injection of hydroxypropyl-beta-cyclodextrin","volume":"70","author":"Nusca","year":"2014","journal-title":"Neurobiol. Dis."},{"key":"10.1016\/j.gene.2017.12.006_bb0180","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1186\/s13023-015-0348-0","article-title":"Visual evoked potentials of Niemann-Pick type C1 mice reveal an impairment of the visual pathway that is rescued by 2-hydroxypropyl-\u00df-cyclodextrin","volume":"10","author":"Palladino","year":"2015","journal-title":"Orphanet J. Rare Dis."},{"key":"10.1016\/j.gene.2017.12.006_bb0185","doi-asserted-by":"crossref","first-page":"400","DOI":"10.1016\/j.bbrc.2011.08.096","article-title":"Npc1 deficiency in the C57BL\/6J genetic background enhances Niemann-Pick disease type C spleen pathology","volume":"413","author":"Parra","year":"2011","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.gene.2017.12.006_bb0190","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.gene.2017.12.006_bb0195","doi-asserted-by":"crossref","first-page":"2193","DOI":"10.1016\/j.bbadis.2014.08.005","article-title":"Excessive microglial activation aggravates olfactory dysfunction by impeding the survival of newborn neurons in the olfactory bulb of Niemann-Pick disease type C1 mice","volume":"1842","author":"Seo","year":"2014","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.gene.2017.12.006_bb0200","doi-asserted-by":"crossref","first-page":"2291","DOI":"10.1002\/glia.23077","article-title":"Cathepsin S contributes to microglia-mediated olfactory dysfunction through the regulation of Cx3cl1-Cx3cr1 axis in a Niemann-pick diseasae type C1 model","volume":"64","author":"Seo","year":"2016","journal-title":"Glia"},{"key":"10.1016\/j.gene.2017.12.006_bb0205","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/S0166-4328(01)00380-1","article-title":"Cognitive deficit and development of motor impairment in a mouse model of Niemann-Pick type C disease","volume":"132","author":"Voikar","year":"2002","journal-title":"Behav. Brain Res."},{"key":"10.1016\/j.gene.2017.12.006_bb0210","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1186\/s12868-014-0126-2","article-title":"Defects in the retina of Niemann-pick type C 1 mutant mice","volume":"15","author":"Yan","year":"2014","journal-title":"BMC Neurosci."},{"key":"10.1016\/j.gene.2017.12.006_bb0215","article-title":"Npc1 acting in neurons and glia is essential for the formation and maintenance of CNS myelin","volume":"4","author":"Yu","year":"2013","journal-title":"PLoS Genet."},{"key":"10.1016\/j.gene.2017.12.006_bb0220","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1007\/s003350010013","article-title":"A modifier of Niemann-Pick C 1 maps to mouse chromosome 19","volume":"11","author":"Zhang","year":"2000","journal-title":"Mamm. Genome"},{"key":"10.1016\/j.gene.2017.12.006_bb0225","doi-asserted-by":"crossref","first-page":"2848","DOI":"10.1002\/jnr.21730","article-title":"Astrocyte-only Npc1 reduces neuronal cholesterol and triples life span of Npc1\u2212\/\u2212 mice","volume":"86","author":"Zhang","year":"2008","journal-title":"J. Neurosci. Res."}],"container-title":["Gene"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0378111917310508?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0378111917310508?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2019,10,7]],"date-time":"2019-10-07T17:04:06Z","timestamp":1570467846000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0378111917310508"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,2]]},"references-count":46,"alternative-id":["S0378111917310508"],"URL":"https:\/\/doi.org\/10.1016\/j.gene.2017.12.006","relation":{},"ISSN":["0378-1119"],"issn-type":[{"value":"0378-1119","type":"print"}],"subject":[],"published":{"date-parts":[[2018,2]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"In Niemann-Pick C1 mouse models, glial-only expression of the normal gene extends survival much further than do changes in genetic background or treatment with hydroxypropyl-beta-cyclodextrin","name":"articletitle","label":"Article Title"},{"value":"Gene","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.gene.2017.12.006","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2017 Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}