{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T03:21:17Z","timestamp":1788837677926,"version":"build-2803163510"},"reference-count":40,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2014,4,1]],"date-time":"2014-04-01T00:00:00Z","timestamp":1396310400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"crossref","award":["ME 1494\/2-1"],"award-info":[{"award-number":["ME 1494\/2-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neurobiology of Disease"],"published-print":{"date-parts":[[2014,4]]},"DOI":"10.1016\/j.nbd.2013.12.018","type":"journal-article","created":{"date-parts":[[2014,1,9]],"date-time":"2014-01-09T04:45:27Z","timestamp":1389242727000},"page":"88-97","source":"Crossref","is-referenced-by-count":41,"special_numbering":"C","title":["The autophagic defect in Niemann\u2013Pick disease type C neurons differs from somatic cells and reduces neuronal viability"],"prefix":"10.1016","volume":"64","author":[{"given":"Volker","family":"Meske","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer","family":"Erz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Timm","family":"Priesnitz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas-Georg","family":"Ohm","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.nbd.2013.12.018_bb0005","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1128\/MCB.06159-11","article-title":"Role of AMPK-mTOR-Ulk1\/2 in the regulation of autophagy: cross talk, shortcuts, and feedbacks","volume":"32","author":"Alers","year":"2012","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.nbd.2013.12.018_bb0010","doi-asserted-by":"crossref","first-page":"37415","DOI":"10.1074\/jbc.M110.186411","article-title":"Macroautophagy is not directly involved in the metabolism of amyloid precursor protein","volume":"285","author":"Boland","year":"2010","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2013.12.018_bb0015","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.nbd.2013.12.018_bb0025","doi-asserted-by":"crossref","first-page":"867","DOI":"10.1016\/j.neuroscience.2004.09.065","article-title":"Pathological cholesterol metabolism fails to modify electrophysiological properties of afflicted neurones in Niemann\u2013Pick disease type C","volume":"130","author":"Deisz","year":"2005","journal-title":"Neuroscience"},{"key":"10.1016\/j.nbd.2013.12.018_bb0030","doi-asserted-by":"crossref","first-page":"5253","DOI":"10.1021\/pr900583m","article-title":"Mapping the phosphorylation sites of Ulk","volume":"8","author":"Dorsey","year":"2009","journal-title":"J. Proteome Res."},{"key":"10.1016\/j.nbd.2013.12.018_bb0035","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1007\/BF00690966","article-title":"Niemann\u2013Pick disease type C. Study on the nature of the cerebral storage process","volume":"66","author":"Elleder","year":"1985","journal-title":"Acta Neuropathol. (Berl)"},{"key":"10.1016\/j.nbd.2013.12.018_bb0040","doi-asserted-by":"crossref","first-page":"4876","DOI":"10.1093\/hmg\/dds324","article-title":"Impaired proteolysis underlies autophagic dysfunction in Niemann\u2013Pick type C disease","volume":"21","author":"Elrick","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2013.12.018_bb0045","doi-asserted-by":"crossref","first-page":"12297","DOI":"10.1074\/jbc.M900573200","article-title":"ULK1_ATG13_FIP200 complex mediates mTOR signaling and is essential for autophagy","volume":"284","author":"Ganley","year":"2009","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2013.12.018_bb0050","doi-asserted-by":"crossref","first-page":"415","DOI":"10.1002\/cne.1149","article-title":"Selective neurodegeneration, without neurofibrillary tangles, in a mouse model of Niemann\u2013Pick C disease","volume":"433","author":"German","year":"2001","journal-title":"J. Comp. Neurol."},{"key":"10.1016\/j.nbd.2013.12.018_bb0055","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1016\/j.molcel.2008.03.003","article-title":"AMPK phosphorylation of raptor mediates a metabolic checkpoint","volume":"30","author":"Gwinn","year":"2008","journal-title":"Mol. Cell"},{"key":"10.1016\/j.nbd.2013.12.018_bb0060","doi-asserted-by":"crossref","first-page":"885","DOI":"10.1038\/nature04724","article-title":"Suppression of basal autophagy in neural cells causes neurodegenerative disease in mice","volume":"441","author":"Hara","year":"2006","journal-title":"Nature"},{"key":"10.1016\/j.nbd.2013.12.018_bb0065","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1083\/jcb.200712064","article-title":"FIP200, a ULK interacting protein, is required for autophagosome formation in mammalian cells","volume":"181","author":"Hara","year":"2008","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2013.12.018_bb0075","doi-asserted-by":"crossref","first-page":"1981","DOI":"10.1091\/mbc.E08-12-1248","article-title":"Nutrient-dependent mTORC1 association with the ULK1-Atg13-FIP200 complex required for autophagy","volume":"20","author":"Hosokawa","year":"2009","journal-title":"Mol. Biol. Cell"},{"key":"10.1016\/j.nbd.2013.12.018_bb0085","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1016\/S0092-8674(03)00929-2","article-title":"TSC2 mediates cellular energy response to control cell growth and survival","volume":"115","author":"Inoki","year":"2003","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2013.12.018_bb0080","doi-asserted-by":"crossref","first-page":"954","DOI":"10.1016\/j.jocn.2008.09.020","article-title":"Association of autophagy with cholesterol-accumulated compartments in Niemann\u2013Pick disease type C cells","volume":"16","author":"Ishibashi","year":"2009","journal-title":"J. Clin. Neurosci."},{"key":"10.1016\/j.nbd.2013.12.018_bb0090","doi-asserted-by":"crossref","first-page":"1992","DOI":"10.1091\/mbc.E08-12-1249","article-title":"ULK-Atg13-FIP200 complexes mediate mTOR signaling to the autophagy machinery","volume":"20","author":"Jung","year":"2009","journal-title":"Mol. Biol. Cell"},{"key":"10.1016\/j.nbd.2013.12.018_bb0095","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1038\/cdd.2010.191","article-title":"The Beclin 1 network regulates autophagy and apoptosis. Review","volume":"18","author":"Kang","year":"2011","journal-title":"Cell Death Differ."},{"key":"10.1016\/j.nbd.2013.12.018_bb0100","doi-asserted-by":"crossref","first-page":"132","DOI":"10.1038\/ncb2152","article-title":"AMPK and mTOR regulate autophagy through direct phosphorylation of Ulk1","volume":"13","author":"Kim","year":"2011","journal-title":"Nat. Cell Biol."},{"key":"10.1016\/j.nbd.2013.12.018_bb0110","doi-asserted-by":"crossref","first-page":"151","DOI":"10.4161\/auto.5338","article-title":"Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes","volume":"4","author":"Klionsky","year":"2008","journal-title":"Autophagy"},{"key":"10.1016\/j.nbd.2013.12.018_bb0115","doi-asserted-by":"crossref","first-page":"849","DOI":"10.4161\/auto.6845","article-title":"Does bafilomycin A1 block the fusion of autophagosomes with lysosomes?","volume":"4","author":"Klionsky","year":"2008","journal-title":"Autophagy"},{"key":"10.1016\/j.nbd.2013.12.018_bb0120","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.nbd.2013.12.018_bb0125","doi-asserted-by":"crossref","first-page":"880","DOI":"10.1038\/nature04723","article-title":"Loss of autophagy in the central nervous system causes neurodegeneration in mice","volume":"441","author":"Komatsu","year":"2006","journal-title":"Nature"},{"key":"10.1016\/j.nbd.2013.12.018_bb0135","doi-asserted-by":"crossref","first-page":"1562","DOI":"10.4161\/auto.7.12.17956","article-title":"Primary lysosomal dysfunction causes cargo-specific deficits of axonal transport leading to Alzheimer-like neuritic dystrophy","volume":"7","author":"Lee","year":"2011","journal-title":"Autophagy"},{"key":"10.1016\/j.nbd.2013.12.018_bb0140","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.nbd.2013.12.018_bb0145","doi-asserted-by":"crossref","first-page":"100","DOI":"10.1074\/jbc.M704292200","article-title":"Coupling of mammalian target of rapamycin with phosphoinositide 3-kinase signaling pathway regulates protein phosphatase 2A- and glycogen synthase kinase-3-dependent phosphorylation of Tau","volume":"283","author":"Meske","year":"2008","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2013.12.018_bb0150","doi-asserted-by":"crossref","first-page":"542","DOI":"10.4161\/auto.4600","article-title":"How to interpret LC3 immunoblotting","volume":"3","author":"Mizushima","year":"2007","journal-title":"Autophagy"},{"key":"10.1016\/j.nbd.2013.12.018_bb0155","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1016\/0022-1759(83)90303-4","article-title":"Rapid colorimetric assay for cellular growth and survival: application to proliferation and cytotoxicity assays","volume":"65","author":"Mosmann","year":"1983","journal-title":"J. Immunol. Methods"},{"key":"10.1016\/j.nbd.2013.12.018_bb0160","doi-asserted-by":"crossref","first-page":"2298","DOI":"10.1126\/science.290.5500.2298","article-title":"Identification of HE1 as the second gene of Niemann\u2013Pick C disease","volume":"290","author":"Naureckiene","year":"2000","journal-title":"Science"},{"key":"10.1016\/j.nbd.2013.12.018_bb0165","doi-asserted-by":"crossref","first-page":"2651","DOI":"10.1093\/hmg\/dds090","article-title":"Disruption and therapeutic rescue of autophagy in a human neuronal model of Niemann Pick type C1","volume":"21","author":"Ordonez","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.nbd.2013.12.018_bb0170","doi-asserted-by":"crossref","first-page":"487","DOI":"10.4161\/auto.4586","article-title":"Lipid trafficking defects increase Beclin-1 and activate autophagy in Niemann\u2013Pick Type C disease","volume":"3","author":"Pacheco","year":"2007","journal-title":"Autophagy"},{"key":"10.1016\/j.nbd.2013.12.018_bb0175","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.nbd.2013.12.018_bb0180","doi-asserted-by":"crossref","first-page":"927","DOI":"10.1016\/j.cell.2005.07.002","article-title":"Bcl-2 antiapoptotic proteins inhibit Beclin 1-dependent autophagy","volume":"122","author":"Pattingre","year":"2005","journal-title":"Cell"},{"key":"10.1016\/j.nbd.2013.12.018_bb0185","doi-asserted-by":"crossref","first-page":"3082","DOI":"10.1128\/MCB.05565-11","article-title":"AMPK, ULK1, autophagy. Review","volume":"31","author":"Roach","year":"2011","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.nbd.2013.12.018_bb0190","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1097\/00005072-198805000-00008","article-title":"Cerebellar involvement in murine sphingomyelinosis: a new model of Niemann\u2013Pick disease","volume":"47","author":"Tanaka","year":"1988","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.nbd.2013.12.018_bb0195","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.nbd.2013.12.018_bb0200","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1212\/WNL.0b013e3181e6210e","article-title":"White and gray matter alterations in adults with Niemann\u2013Pick disease type C: a cross-sectional study","volume":"75","author":"Walterfang","year":"2010","journal-title":"Neurology"},{"key":"10.1016\/j.nbd.2013.12.018_bb0205","doi-asserted-by":"crossref","first-page":"4764","DOI":"10.1073\/pnas.0910872107","article-title":"Cholesterol trafficking is required for mTOR activation in endothelial cells","volume":"107","author":"Xu","year":"2010","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.nbd.2013.12.018_bb0210","doi-asserted-by":"crossref","first-page":"288","DOI":"10.1016\/S0387-7604(01)00209-1","article-title":"Progressive neuronal loss in the ventral posterior lateral and medial nuclei of thalamus in Niemann\u2013Pick disease type C mouse brain","volume":"23","author":"Yamada","year":"2001","journal-title":"Brain Dev."},{"key":"10.1016\/j.nbd.2013.12.018_bb0215","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1247\/csf.23.33","article-title":"Bafilomycin A1 prevents maturation of autophagic vacuoles by inhibiting fusion between autophagosomes and lysosomes in rat hepatoma cell line, H-4-II-E cells","volume":"23","author":"Yamamoto","year":"1998","journal-title":"Cell Struct. Funct."},{"key":"10.1016\/j.nbd.2013.12.018_bb0220","doi-asserted-by":"crossref","first-page":"616","DOI":"10.4161\/auto.5.5.8091","article-title":"Tap42-associated protein phosphatase type 2A negatively regulates induction of autophagy","volume":"5","author":"Yorimitsu","year":"2009","journal-title":"Autophagy"}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996114000059?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0969996114000059?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2018,10,7]],"date-time":"2018-10-07T08:23:42Z","timestamp":1538900622000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0969996114000059"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2014,4]]},"references-count":40,"alternative-id":["S0969996114000059"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2013.12.018","relation":{},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2014,4]]}}}