{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T17:10:19Z","timestamp":1791220219473,"version":"4.1.0"},"reference-count":48,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2009,5,1]],"date-time":"2009-05-01T00:00:00Z","timestamp":1241136000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Research"],"published-print":{"date-parts":[[2009,5]]},"DOI":"10.1016\/j.brainres.2009.03.027","type":"journal-article","created":{"date-parts":[[2009,3,26]],"date-time":"2009-03-26T05:09:22Z","timestamp":1238044162000},"page":"140-151","source":"Crossref","is-referenced-by-count":59,"special_numbering":"C","title":["Allopregnanolone treatment delays cholesterol accumulation and reduces autophagic\/lysosomal dysfunction and inflammation in Npc1\u2212\/\u2212 mouse brain"],"prefix":"10.1016","volume":"1270","author":[{"given":"Guanghong","family":"Liao","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Cheung","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Galeano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angela X.","family":"Ji","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qingyu","family":"Qin","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoning","family":"Bi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.brainres.2009.03.027_bib1","doi-asserted-by":"crossref","first-page":"811","DOI":"10.1002\/jnr.20685","article-title":"Allopregnanolone treatment, both as a single injection or repetitively, delays demyelination and enhances survival of Niemann\u2013Pick C mice","volume":"82","author":"Ahmad","year":"2005","journal-title":"J. Neurosci. Res."},{"key":"10.1016\/j.brainres.2009.03.027_bib2","first-page":"25","article-title":"Apoptosis and autophagy in nigral neurons of patients with Parkinson's disease","volume":"12","author":"Anglade","year":"1997","journal-title":"Histol. Histopathol."},{"key":"10.1016\/j.brainres.2009.03.027_bib3","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1007\/BF00318566","article-title":"Paired helical filament tau (PHFtau) in Niemann\u2013Pick type C disease is similar to PHFtau in Alzheimer's disease","volume":"90","author":"Auer","year":"1995","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.brainres.2009.03.027_bib4","doi-asserted-by":"crossref","first-page":"887","DOI":"10.1016\/S0014-4886(03)00345-5","article-title":"Postnatal development of inflammation in a murine model of Niemann\u2013Pick type C disease: immunohistochemical observations of microglia and astroglia","volume":"184","author":"Baudry","year":"2003","journal-title":"Exp. Neurol."},{"key":"10.1016\/j.brainres.2009.03.027_bib5","doi-asserted-by":"crossref","first-page":"646","DOI":"10.4161\/auto.5074","article-title":"Autophagic\u2013lysosomal dysfunction and neurodegeneration in Niemann\u2013Pick type C mice: lipid starvation or indigestion?","volume":"3","author":"Bi","year":"2007","journal-title":"Autophagy"},{"key":"10.1016\/j.brainres.2009.03.027_bib6","doi-asserted-by":"crossref","first-page":"1469","DOI":"10.1046\/j.1471-4159.2000.0741469.x","article-title":"Novel cathepsin D inhibitors block the formation of hyperphosphorylated tau fragments in hippocampus","volume":"74","author":"Bi","year":"2000","journal-title":"J. Neurochem."},{"key":"10.1016\/j.brainres.2009.03.027_bib7","doi-asserted-by":"crossref","first-page":"1081","DOI":"10.1016\/S0002-9440(10)61197-2","article-title":"Deregulation of the phosphatidylinositol-3 kinase signaling cascade is associated with neurodegeneration in Npc1\u2212\/\u2212 mouse brain","volume":"167","author":"Bi","year":"2005","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.brainres.2009.03.027_bib8","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1038\/nrn2038","article-title":"Microglia-mediated neurotoxicity: uncovering the molecular mechanisms","volume":"8","author":"Block","year":"2007","journal-title":"Nat. Rev. Neurosci."},{"key":"10.1016\/j.brainres.2009.03.027_bib9","doi-asserted-by":"crossref","first-page":"789","DOI":"10.1016\/j.cell.2006.06.049","article-title":"Anatomical profiling of nuclear receptor expression reveals a hierarchical transcriptional network","volume":"126","author":"Bookout","year":"2006","journal-title":"Cell"},{"key":"10.1016\/j.brainres.2009.03.027_bib10","first-page":"110","article-title":"Staining of cholesterol with the fluorescent antibiotic \u201cfilipin\u201d","volume":"50","author":"Bornig","year":"1974","journal-title":"Acta Histochem."},{"key":"10.1016\/j.brainres.2009.03.027_bib11","doi-asserted-by":"crossref","first-page":"556","DOI":"10.1073\/pnas.84.2.556","article-title":"Niemann\u2013Pick variant disorders: comparison of errors of cellular cholesterol homeostasis in group D and group C fibroblasts","volume":"84","author":"Butler","year":"1987","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.brainres.2009.03.027_bib12","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1126\/science.277.5323.228","article-title":"Niemann\u2013Pick C1 disease gene: homology to mediators of cholesterol homeostasis","volume":"277","author":"Carstea","year":"1997","journal-title":"Science"},{"key":"10.1016\/j.brainres.2009.03.027_bib13","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1016\/S0306-4522(01)00517-6","article-title":"Neurodegeneration in the Niemann\u2013Pick C mouse: glial involvement","volume":"109","author":"German","year":"2002","journal-title":"Neuroscience"},{"key":"10.1016\/j.brainres.2009.03.027_bib14","doi-asserted-by":"crossref","first-page":"405","DOI":"10.1016\/j.cell.2007.09.012","article-title":"Systematic gene expression mapping clusters nuclear receptors according to their function in the brain","volume":"131","author":"Gofflot","year":"2007","journal-title":"Cell"},{"key":"10.1016\/j.brainres.2009.03.027_bib15","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.brainres.2009.03.027_bib16","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1007\/BF00227765","article-title":"Cerebellar degeneration in the Niemann\u2013Pick type C mouse","volume":"85","author":"Higashi","year":"1993","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.brainres.2009.03.027_bib17","doi-asserted-by":"crossref","first-page":"5720","DOI":"10.1093\/emboj\/19.21.5720","article-title":"LC3, a mammalian homologue of yeast Apg8p, is localized in autophagosome membranes after processing","volume":"19","author":"Kabeya","year":"2000","journal-title":"EMBO J."},{"key":"10.1016\/j.brainres.2009.03.027_bib18","doi-asserted-by":"crossref","first-page":"1805","DOI":"10.1523\/JNEUROSCI.4599-08.2009","article-title":"Hippocampal and cognitive aging across the lifespan: a bioenergetic shift precedes and increased cholesterol trafficking parallels memory impairment","volume":"29","author":"Kadish","year":"2009","journal-title":"J. Neurosci."},{"key":"10.1016\/j.brainres.2009.03.027_bib19","first-page":"81","article-title":"Cell-autonomous death of cerebellar Purkinje neurons with autophagy in Niemann\u2013Pick type C disease","volume":"1","author":"Ko","year":"2005","journal-title":"PLoS Genet."},{"key":"10.1016\/j.brainres.2009.03.027_bib20","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.brainres.2009.03.027_bib21","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1186\/1742-2094-5-8","article-title":"Neuroinflammation mediated by IL-1beta increases susceptibility of dopamine neurons to degeneration in an animal model of Parkinson's disease","volume":"5","author":"Koprich","year":"2008","journal-title":"J. Neuroinflammation."},{"key":"10.1016\/j.brainres.2009.03.027_bib22","doi-asserted-by":"crossref","first-page":"13807","DOI":"10.1073\/pnas.0606218103","article-title":"Pregnane X receptor (PXR) activation: a mechanism for neuroprotection in a mouse model of Niemann\u2013Pick C disease","volume":"103","author":"Langmade","year":"2006","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.brainres.2009.03.027_bib23","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1093\/jnen\/64.4.323","article-title":"Molecular, anatomical, and biochemical events associated with neurodegeneration in mice with Niemann\u2013Pick type C disease","volume":"64","author":"Li","year":"2005","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.brainres.2009.03.027_bib24","doi-asserted-by":"crossref","first-page":"962","DOI":"10.2353\/ajpath.2007.070052","article-title":"Cholesterol accumulation is associated with lysosomal dysfunction and autophagic stress in Npc1\u2212\/\u2212 mouse brain","volume":"171","author":"Liao","year":"2007","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.brainres.2009.03.027_bib25","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1194\/jlr.M700525-JLR200","article-title":"Genetic variations and treatments that affect the lifespan of the NPC1 mouse","volume":"49","author":"Liu","year":"2008","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.brainres.2009.03.027_bib26","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.brainres.2009.03.027_bib27","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1007\/s004010050689","article-title":"GABAergic neuroaxonal dystrophy and other cytopathological alterations in feline Niemann\u2013Pick disease type C","volume":"94","author":"March","year":"1997","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.brainres.2009.03.027_bib28","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1016\/j.pharmthera.2007.04.011","article-title":"Neurosteroid regulation of central nervous system development","volume":"116","author":"Mellon","year":"2007","journal-title":"Pharmacol. Ther."},{"key":"10.1016\/j.brainres.2009.03.027_bib29","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1093\/jnen\/64.2.113","article-title":"Extensive involvement of autophagy in Alzheimer disease: an immuno-electron microscopy study","volume":"64","author":"Nixon","year":"2005","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.brainres.2009.03.027_bib30","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1007\/s002210100870","article-title":"Neurodegeneration in Niemann\u2013Pick type C disease mice","volume":"141","author":"Ong","year":"2001","journal-title":"Exp. Brain Res."},{"key":"10.1016\/j.brainres.2009.03.027_bib31","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1023\/A:1023993405851","article-title":"Apolipoprotein D in the Niemann\u2013Pick type C disease mouse brain: an ultrastructural immunocytochemical analysis","volume":"31","author":"Ong","year":"2002","journal-title":"J. Neurocytol."},{"key":"10.1016\/j.brainres.2009.03.027_bib32","doi-asserted-by":"crossref","first-page":"1495","DOI":"10.1093\/hmg\/ddm100","article-title":"Autophagy in Niemann\u2013Pick C disease is dependent upon Beclin-1 and responsive to lipid trafficking defects","volume":"16","author":"Pacheco","year":"2007","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.brainres.2009.03.027_bib33","doi-asserted-by":"crossref","first-page":"1250","DOI":"10.1046\/j.1471-4159.2000.0751250.x","article-title":"Regional and developmental expression of the Npc1 mRNA in the mouse brain","volume":"75","author":"Prasad","year":"2000","journal-title":"J. Neurochem."},{"key":"10.1016\/j.brainres.2009.03.027_bib34","doi-asserted-by":"crossref","first-page":"582","DOI":"10.1194\/jlr.D300032-JLR200","article-title":"A novel cholesterol stain reveals early neuronal cholesterol accumulation in the Niemann\u2013Pick type C1 mouse brain","volume":"45","author":"Reid","year":"2004","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.brainres.2009.03.027_bib35","doi-asserted-by":"crossref","first-page":"14470","DOI":"10.1523\/JNEUROSCI.4823-07.2007","article-title":"Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse","volume":"27","author":"Repa","year":"2007","journal-title":"J. Neurosci."},{"key":"10.1016\/j.brainres.2009.03.027_bib36","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1016\/j.bbalip.2004.08.006","article-title":"The NPC1 protein: structure implies function","volume":"1685","author":"Scott","year":"2004","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.brainres.2009.03.027_bib37","doi-asserted-by":"crossref","first-page":"2179","DOI":"10.2174\/092986706777935203","article-title":"Towards a unifying hypothesis of Alzheimer's disease: cholinergic system linked to plaques, tangles and neuroinflammation","volume":"13","author":"Sivaprakasam","year":"2006","journal-title":"Curr. Med. Chem."},{"key":"10.1016\/j.brainres.2009.03.027_bib38","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1046\/j.1471-4159.1998.70010242.x","article-title":"Cellular cholesterol storage in the Niemann\u2013Pick disease type C mouse is associated with increased expression and defective processing of apolipoprotein D","volume":"70","author":"Suresh","year":"1998","journal-title":"J. Neurochem."},{"key":"10.1016\/j.brainres.2009.03.027_bib39","doi-asserted-by":"crossref","first-page":"227","DOI":"10.1007\/BF00309338","article-title":"Neurofibrillary tangles in Niemann\u2013Pick disease type C","volume":"89","author":"Suzuki","year":"1995","journal-title":"Acta. Neuropathol."},{"key":"10.1016\/j.brainres.2009.03.027_bib40","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1007\/s12035-008-8024-1","article-title":"Neurosteroids in the Purkinje cell: biosynthesis, mode of action and functional significance","volume":"37","author":"Tsutsui","year":"2008","journal-title":"Mol. Neurobiol."},{"key":"10.1016\/j.brainres.2009.03.027_bib41","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1080\/14734220310016169","article-title":"A novel aspect of the cerebellum: biosynthesis of neurosteroids in the Purkinje cell","volume":"2","author":"Tsutsui","year":"2003","journal-title":"Cerebellum"},{"key":"10.1016\/j.brainres.2009.03.027_bib42","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1111\/j.1399-0004.1988.tb03460.x","article-title":"Niemann\u2013Pick disease group C: clinical variability and diagnosis based on defective cholesterol esterification. A collaborative study on 70 patients","volume":"33","author":"Vanier","year":"1988","journal-title":"Clin. Genet."},{"key":"10.1016\/j.brainres.2009.03.027_bib43","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1023\/A:1022575511354","article-title":"Lipid changes in Niemann\u2013Pick disease type C brain: personal experience and review of the literature","volume":"24","author":"Vanier","year":"1999","journal-title":"Neurochem. Res."},{"key":"10.1016\/j.brainres.2009.03.027_bib44","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.bbalip.2004.08.011","article-title":"Consequences of NPC1 and NPC2 loss of function in mammalian neurons","volume":"1685","author":"Walkley","year":"2004","journal-title":"Biochim. Biophys. Acta."},{"key":"10.1016\/j.brainres.2009.03.027_bib45","first-page":"E336","article-title":"Cholesterol balance and metabolism in mice with loss of function of Niemann\u2013Pick C protein","volume":"276","author":"Xie","year":"1999","journal-title":"Am. J. Physiol."},{"key":"10.1016\/j.brainres.2009.03.027_bib46","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1093\/jnen\/60.1.49","article-title":"Neurons in Niemann\u2013Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations","volume":"60","author":"Zervas","year":"2001","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.brainres.2009.03.027_bib47","doi-asserted-by":"crossref","first-page":"1283","DOI":"10.1016\/S0960-9822(01)00396-7","article-title":"Critical role for glycosphingolipids in Niemann\u2013Pick disease type C","volume":"11","author":"Zervas","year":"2001","journal-title":"Curr. Biol."},{"key":"10.1016\/j.brainres.2009.03.027_bib48","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":["Brain Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0006899309005599?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0006899309005599?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2018,12,20]],"date-time":"2018-12-20T16:56:41Z","timestamp":1545325001000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0006899309005599"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,5]]},"references-count":48,"alternative-id":["S0006899309005599"],"URL":"https:\/\/doi.org\/10.1016\/j.brainres.2009.03.027","relation":{},"ISSN":["0006-8993"],"issn-type":[{"value":"0006-8993","type":"print"}],"subject":[],"published":{"date-parts":[[2009,5]]}}}