{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T17:53:15Z","timestamp":1790704395109,"version":"4.1.0"},"reference-count":47,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2003,12,1]],"date-time":"2003-12-01T00:00:00Z","timestamp":1070236800000},"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":["Neurobiology of Disease"],"published-print":{"date-parts":[[2003,12]]},"DOI":"10.1016\/j.nbd.2003.08.016","type":"journal-article","created":{"date-parts":[[2003,12,11]],"date-time":"2003-12-11T12:47:40Z","timestamp":1071146860000},"page":"595-601","source":"Crossref","is-referenced-by-count":88,"title":["Toxicity of glucosylsphingosine (glucopsychosine) to cultured neuronal cells: a model system for assessing neuronal damage in Gaucher disease type 2 and 3"],"prefix":"10.1016","volume":"14","author":[{"given":"U.H","family":"Schueler","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T","family":"Kolter","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.R","family":"Kaneski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.K","family":"Blusztajn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Herkenham","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K","family":"Sandhoff","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.O","family":"Brady","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.nbd.2003.08.016_BIB1","doi-asserted-by":"crossref","first-page":"1464","DOI":"10.1056\/NEJM199105233242104","article-title":"Replacement therapy for inherited enzyme deficiency\u2014macrophage-targeted glucocerebrosidase for Gaucher's disease","volume":"324","author":"Barton","year":"1991","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.nbd.2003.08.016_BIB2","doi-asserted-by":"crossref","first-page":"1814","DOI":"10.1096\/fj.02-0149fje","article-title":"Phosphatidylcholine synthesis is elevated in neuronal models of Gaucher disease due to direct activation of CTP","volume":"16","author":"Bodennec","year":"2002","journal-title":"FASEB J."},{"key":"10.1016\/j.nbd.2003.08.016_BIB3","doi-asserted-by":"crossref","first-page":"1112","DOI":"10.1172\/JCI105417","article-title":"Demonstration of a deficiency of glucocerebroside-cleaving enzyme in Gaucher's disease","volume":"45","author":"Brady","year":"1966","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.nbd.2003.08.016_BIB4","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1016\/0006-291X(65)90743-6","article-title":"Metabolism of glucocerebrosides. II. Evidence of an enzymatic deficiency in Gaucher's disease","volume":"18","author":"Brady","year":"1965","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.nbd.2003.08.016_BIB5","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1007\/BF02533018","article-title":"Enzymatic synthesis of glucosylsphingosine by rat brain microsomes","volume":"7","author":"Curtino","year":"1972","journal-title":"Lipids"},{"key":"10.1016\/j.nbd.2003.08.016_BIB6","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1055\/s-2008-1071549","article-title":"Enzyme replacement therapy of infantile Gaucher disease","volume":"24","author":"Erikson","year":"1993","journal-title":"Neuropediatrics"},{"key":"10.1016\/j.nbd.2003.08.016_BIB7","unstructured":"Folch, J., Lees, M., Sloane-Stanley, G., (1957). A simple method for the isolation and purification of total lipids from animal tissues. J. Biol. Chem., 497\u2013509"},{"key":"10.1016\/j.nbd.2003.08.016_BIB8","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1016\/0009-3084(76)90015-3","article-title":"Synthesis of 2-n-(hexadecanoyl)-amino-4-nitrophenyl phosphorylcholine-hydroxide, a chromogenic substrate for assaying sphingomyelinase activity","volume":"16","author":"Gal","year":"1976","journal-title":"Chem. Phys. Lipids"},{"key":"10.1016\/j.nbd.2003.08.016_BIB9","doi-asserted-by":"crossref","first-page":"109","DOI":"10.1159\/000469416","article-title":"Use of activators and inhibitors to define the properties of the active site of normal and Gaucher disease lysosomal beta-glucosidase","volume":"33","author":"Gatt","year":"1985","journal-title":"Enzyme"},{"key":"10.1016\/j.nbd.2003.08.016_BIB10","doi-asserted-by":"crossref","first-page":"300","DOI":"10.1002\/ana.410230315","article-title":"Infantile Gaucher's disease","volume":"23","author":"Grafe","year":"1988","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2003.08.016_BIB11","doi-asserted-by":"crossref","first-page":"670","DOI":"10.1126\/science.3101176","article-title":"Lysosphingolipids inhibit protein kinase C","volume":"235","author":"Hannun","year":"1987","journal-title":"Science"},{"key":"10.1016\/j.nbd.2003.08.016_BIB12","doi-asserted-by":"crossref","first-page":"500","DOI":"10.1126\/science.2643164","article-title":"Functions of sphingolipids and sphingolipid breakdown products in cellular regulation","volume":"243","author":"Hannun","year":"1989","journal-title":"Science"},{"key":"10.1016\/j.nbd.2003.08.016_BIB13","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1023\/A:1020259630034","article-title":"Cationic glycosphingolipids in neuronal tissues and their possible biological significance","volume":"27","author":"Hikita","year":"2002","journal-title":"Neurochem. Res."},{"key":"10.1016\/j.nbd.2003.08.016_BIB14","doi-asserted-by":"crossref","first-page":"429","DOI":"10.1083\/jcb.153.2.429","article-title":"Identification of a molecular target of psychosine and its role in globoid cell formation","volume":"153","author":"Im","year":"2001","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.nbd.2003.08.016_BIB15","doi-asserted-by":"crossref","first-page":"25859","DOI":"10.1074\/jbc.R200001200","article-title":"Combinatorial ganglioside biosynthesis","volume":"277","author":"Kolter","year":"2002","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2003.08.016_BIB16","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1111\/j.1750-3639.1998.tb00138.x","article-title":"Recent advances in the biochemistry of sphingolipidoses","volume":"8","author":"Kolter","year":"1998","journal-title":"Brain Pathol."},{"key":"10.1016\/j.nbd.2003.08.016_BIB17","doi-asserted-by":"crossref","first-page":"3868","DOI":"10.1016\/S0021-9258(19)39674-7","article-title":"Activation of phospholipase D by sphingoid bases in NG108\u201315 neural-derived cells","volume":"265","author":"Lavie","year":"1990","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2003.08.016_BIB18","doi-asserted-by":"crossref","first-page":"10086","DOI":"10.1073\/pnas.90.21.10086","article-title":"Phospholipase D-catalyzed hydrolysis of phosphatidylcholine provides the choline precursor for acetylcholine synthesis in a human neuronal cell line","volume":"90","author":"Lee","year":"1993","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"10.1016\/j.nbd.2003.08.016_BIB19","doi-asserted-by":"crossref","first-page":"1762","DOI":"10.1111\/j.1471-4159.1983.tb08153.x","article-title":"Comparative hydrolysis of sphingomyelin and 2-N-(hexadecanoyl)-amino-4-nitrophenyl-phosphorylcholine by normal human brain homogenate at acid and neutral pH","volume":"40","author":"Levade","year":"1983","journal-title":"J. Neurochem."},{"key":"10.1016\/j.nbd.2003.08.016_BIB20","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1515\/bchm3.1986.367.1.241","article-title":"Occurrence of lysoganglioside lyso-GM2 (II3-Neu5Ac-gangliotriaosylsphingosine) in GM2 gangliosidosis brain","volume":"367","author":"Neuenhofer","year":"1986","journal-title":"Biol. Chem. Hoppe Seyler"},{"key":"10.1016\/j.nbd.2003.08.016_BIB21","doi-asserted-by":"crossref","first-page":"709","DOI":"10.1111\/j.1471-4159.1982.tb07950.x","article-title":"Accumulation of glucosylceramide and glucosylsphingosine (psychosine) in cerebrum and cerebellum in infantile and juvenile Gaucher disease","volume":"39","author":"Nilsson","year":"1982","journal-title":"J. Neurochem."},{"key":"10.1016\/j.nbd.2003.08.016_BIB22","doi-asserted-by":"crossref","first-page":"262","DOI":"10.1016\/S1096-7192(02)00117-8","article-title":"Glucosylsphingosine accumulation in tissues from patients with Gaucher disease","volume":"76","author":"Orvisky","year":"2002","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.nbd.2003.08.016_BIB23","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1203\/00006450-200008000-00018","article-title":"Glucosylsphingosine accumulation in mice and patients with type 2 Gaucher disease begins early in gestation","volume":"48","author":"Orvisky","year":"2000","journal-title":"Pediatr. Res."},{"key":"10.1016\/j.nbd.2003.08.016_BIB24","doi-asserted-by":"crossref","first-page":"5256","DOI":"10.1016\/S0021-9258(19)43595-3","article-title":"Isolation and characterization of glucocerebrosidase from human placental tissue","volume":"248","author":"Pentchev","year":"1973","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2003.08.016_BIB25","doi-asserted-by":"crossref","first-page":"569","DOI":"10.1016\/0003-9861(76)90547-6","article-title":"Differentiation of beta-glucocerebrosidase from beta-glucosidase in human tissues using sodium taurocholate","volume":"175","author":"Peters","year":"1976","journal-title":"Arch. Biochem. Biophys."},{"key":"10.1016\/j.nbd.2003.08.016_BIB26","doi-asserted-by":"crossref","first-page":"188","DOI":"10.1111\/j.1471-4159.1983.tb13668.x","article-title":"Acetylcholine and choline in neuronal tissue measured by HPLC with electrochemical detection","volume":"41","author":"Potter","year":"1983","journal-title":"J. Neurochem."},{"key":"10.1016\/j.nbd.2003.08.016_BIB27","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1002\/(SICI)1096-8628(19970711)71:1<16::AID-AJMG3>3.3.CO;2-U","article-title":"Gaucher disease","volume":"71","author":"Prows","year":"1997","journal-title":"Am. J. Med. Genet."},{"key":"10.1016\/j.nbd.2003.08.016_BIB28","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1016\/0006-8993(89)91253-5","article-title":"Acetylcholine synthesis and secretion by LA-N-2 human neuroblastoma cells","volume":"476","author":"Richardson","year":"1989","journal-title":"Brain Res."},{"key":"10.1016\/j.nbd.2003.08.016_BIB29","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1023\/A:1022501702403","article-title":"Sphingosylphosphorylcholine in Niemann-Pick disease brain","volume":"24","author":"Rodriguez-Lafrasse","year":"1999","journal-title":"Neurochem. Res."},{"key":"10.1016\/j.nbd.2003.08.016_BIB30","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1093\/oxfordjournals.jbchem.a124284","article-title":"Glucosylceramide and glucosylsphingosine metabolism in cultured fibroblasts deficient in acid beta-glucosidase activity","volume":"115","author":"Sasagasako","year":"1994","journal-title":"J. Biochem. (Tokyo)"},{"key":"10.1016\/j.nbd.2003.08.016_BIB31","doi-asserted-by":"crossref","first-page":"613","DOI":"10.1002\/ana.410420412","article-title":"Prospective study of neurological responses to treatment with macrophage-targeted glucocerebrosidase in patients with type 3 Gaucher's disease","volume":"42","author":"Schiffmann","year":"1997","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.nbd.2003.08.016_BIB32","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1023\/A:1014915430398","article-title":"Uptake of mannose-terminal glucocerebrosidase in cultured human cholinergic and dopaminergic neuron cell lines","volume":"27","author":"Schueler","year":"2002","journal-title":"Neurochem. Res."},{"key":"10.1016\/j.nbd.2003.08.016_BIB33","first-page":"1364","article-title":"Morphology, growth, chromosomal pattern and fibrinolytic activity of two new human neuroblastoma cell lines","volume":"37","author":"Seeger","year":"1977","journal-title":"Cancer Res."},{"key":"10.1016\/j.nbd.2003.08.016_BIB34","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/0003-2697(85)90442-7","article-title":"Measurement of protein using bicinchoninic acid","volume":"150","author":"Smith","year":"1985","journal-title":"Anal. Biochem."},{"key":"10.1016\/j.nbd.2003.08.016_BIB35","doi-asserted-by":"crossref","first-page":"11746","DOI":"10.1016\/S0021-9258(19)38461-3","article-title":"Sphingosine inhibits the activity of rat liver CTP","volume":"265","author":"Sohal","year":"1990","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2003.08.016_BIB36","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1016\/0076-6879(78)50049-9","article-title":"Enzymic diagnosis of sphingolipidoses","volume":"50","author":"Suzuki","year":"1978","journal-title":"Methods Enzymol."},{"key":"10.1016\/j.nbd.2003.08.016_BIB37","doi-asserted-by":"crossref","first-page":"251","DOI":"10.1023\/A:1022436928925","article-title":"Twenty five years of the \u201cpsychosine hypothesis:\u201d","volume":"23","author":"Suzuki","year":"1998","journal-title":"Neurochem. Res."},{"key":"10.1016\/j.nbd.2003.08.016_BIB38","first-page":"505","article-title":"Sphingomyelin and glucocerebroside of spleen in cases of Gaucher's and Niemann-Pick's disease","volume":"37","author":"Taketomi","year":"1967","journal-title":"Jpn. J. Exp. Med."},{"key":"10.1016\/j.nbd.2003.08.016_BIB39","doi-asserted-by":"crossref","first-page":"490","DOI":"10.1097\/00005072-199309000-00007","article-title":"Effects of psychosine (galactosylsphingosine) on the survival and the fine structure of cultured Schwann cells","volume":"52","author":"Tanaka","year":"1993","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.nbd.2003.08.016_BIB40","doi-asserted-by":"crossref","first-page":"1585","DOI":"10.1111\/j.1471-4159.1990.tb04942.x","article-title":"Lysosulfatide (sulfogalactosylsphingosine) accumulation in tissues from patients with metachromatic leukodystrophy","volume":"55","author":"Toda","year":"1990","journal-title":"J. Neurochem."},{"key":"10.1016\/j.nbd.2003.08.016_BIB41","doi-asserted-by":"crossref","first-page":"30576","DOI":"10.1074\/jbc.270.51.30576","article-title":"pH-dependent conformational properties of saposins and their interactions with phospholipid membranes","volume":"270","author":"Vaccaro","year":"1995","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2003.08.016_BIB42","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1111\/j.1432-1033.1985.tb08655.x","article-title":"Characterization of human glucosylsphingosine glucosyl hydrolase and comparison with glucosylceramidase","volume":"146","author":"Vaccaro","year":"1985","journal-title":"Eur. J. Biochem."},{"key":"10.1016\/j.nbd.2003.08.016_BIB43","doi-asserted-by":"crossref","first-page":"30271","DOI":"10.1074\/jbc.273.46.30271","article-title":"Lysosomal degradation on vesicular membrane surfaces. Enhanced glucosylceramide degradation by lysosomal anionic lipids and activators","volume":"273","author":"Wilkening","year":"1998","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.nbd.2003.08.016_BIB44","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1007\/BF02962142","article-title":"A biochemical and ultrastructural evaluation of the type 2 Gaucher mouse","volume":"24","author":"Willemsen","year":"1995","journal-title":"Mol. Chem. Neuropathol."},{"key":"10.1016\/j.nbd.2003.08.016_BIB45","doi-asserted-by":"crossref","first-page":"994","DOI":"10.1212\/WNL.33.8.994","article-title":"Non-infantile neuronopathic Gaucher's disease","volume":"33","author":"Winkelman","year":"1983","journal-title":"Neurology"},{"key":"10.1016\/j.nbd.2003.08.016_BIB46","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1186\/1471-2202-2-16","article-title":"Generation of choline for acetylcholine synthesis by phospholipase D isoforms","volume":"2","author":"Zhao","year":"2001","journal-title":"BMC Neurosci."},{"key":"10.1016\/j.nbd.2003.08.016_BIB47","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1023\/A:1022578424693","article-title":"Delivery, distribution, and neuronal uptake of exogenous mannose-terminal glucocerebrosidase in the intact rat brain","volume":"24","author":"Zirzow","year":"1999","journal-title":"Neurochem. Res."}],"container-title":["Neurobiology of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S096999610300161X?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S096999610300161X?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2021,6,13]],"date-time":"2021-06-13T13:19:03Z","timestamp":1623590343000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S096999610300161X"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2003,12]]},"references-count":47,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2003,12]]}},"alternative-id":["S096999610300161X"],"URL":"https:\/\/doi.org\/10.1016\/j.nbd.2003.08.016","relation":{},"ISSN":["0969-9961"],"issn-type":[{"value":"0969-9961","type":"print"}],"subject":[],"published":{"date-parts":[[2003,12]]}}}