{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,6]],"date-time":"2026-05-06T03:19:03Z","timestamp":1778037543117,"version":"3.51.4"},"reference-count":39,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2019,2,1]],"date-time":"2019-02-01T00:00:00Z","timestamp":1548979200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2019,2,1]],"date-time":"2019-02-01T00:00:00Z","timestamp":1548979200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2020,2,1]],"date-time":"2020-02-01T00:00:00Z","timestamp":1580515200000},"content-version":"vor","delay-in-days":365,"URL":"http:\/\/www.elsevier.com\/open-access\/userlicense\/1.0\/"}],"funder":[{"DOI":"10.13039\/100007765","name":"Kansas State University","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007765","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease"],"published-print":{"date-parts":[[2019,2]]},"DOI":"10.1016\/j.bbadis.2018.11.011","type":"journal-article","created":{"date-parts":[[2018,11,16]],"date-time":"2018-11-16T12:27:18Z","timestamp":1542371238000},"page":"322-328","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":17,"title":["Neuronal ceroid lipofuscinosis related ER membrane protein CLN8 regulates PP2A activity and ceramide levels"],"prefix":"10.1016","volume":"1865","author":[{"given":"Babita","family":"Adhikari","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bhagya","family":"De Silva","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joshua A.","family":"Molina","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ashton","family":"Allen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sun H.","family":"Peck","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stella Y.","family":"Lee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.bbadis.2018.11.011_bb0005","doi-asserted-by":"crossref","first-page":"535","DOI":"10.1023\/A:1005564509027","article-title":"The neuronal ceroid-lipofuscinoses (Batten disease): a new class of lysosomal storage diseases","volume":"22","author":"Bennett","year":"1999","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0010","doi-asserted-by":"crossref","first-page":"850","DOI":"10.1016\/j.bbadis.2006.06.010","article-title":"The neuronal ceroid-lipofuscinoses: from past to present","volume":"1762","author":"Haltia","year":"2006","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2018.11.011_bb0015","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1212\/WNL.0b013e31825f0547","article-title":"New nomenclature and classification scheme for the neuronal ceroid lipofuscinoses","volume":"79","author":"Williams","year":"2012","journal-title":"Neurology"},{"key":"10.1016\/j.bbadis.2018.11.011_bb0020","doi-asserted-by":"crossref","first-page":"1691","DOI":"10.1093\/hmg\/9.11.1691","article-title":"The neuronal ceroid lipofuscinosis CLN8 membrane protein is a resident of the endoplasmic reticulum","volume":"9","author":"Lonka","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0025","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1016\/S0968-0004(02)02154-0","article-title":"TRAM, LAG1 and CLN8: members of a novel family of lipid-sensing domains?","volume":"27","author":"Winter","year":"2002","journal-title":"Trends Biochem. Sci."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0030","doi-asserted-by":"crossref","first-page":"25001","DOI":"10.1074\/jbc.R600010200","article-title":"When do lasses (longevity assurance genes) become CerS (ceramide synthases)?: insights into the regulation of ceramide synthesis","volume":"281","author":"Pewzner-Jung","year":"2006","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0035","doi-asserted-by":"crossref","first-page":"37083","DOI":"10.1074\/jbc.M307554200","article-title":"Human homologues of LAG1 reconstitute acyl-CoA-dependent ceramide synthesis in yeast","volume":"278","author":"Guillas","year":"2003","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0040","doi-asserted-by":"crossref","first-page":"3798","DOI":"10.1002\/elps.201200472","article-title":"CLN5 and CLN8 protein association with ceramide synthase: biochemical and proteomic approaches","volume":"33","author":"Haddad","year":"2012","journal-title":"Electrophoresis"},{"key":"10.1016\/j.bbadis.2018.11.011_bb0045","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1111\/j.1471-4159.2005.03376.x","article-title":"Mass spectrometric analysis reveals changes in phospholipid, neutral sphingolipid and sulfatide molecular species in progressive epilepsy with mental retardation, EPMR, brain: a case study","volume":"95","author":"Hermansson","year":"2005","journal-title":"J. Neurochem."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0050","doi-asserted-by":"crossref","first-page":"625","DOI":"10.1203\/PDR.0b013e31816fdc17","article-title":"CLN3p impacts galactosylceramide transport, raft morphology, and lipid content","volume":"63","author":"Rusyn","year":"2008","journal-title":"Pediatr. Res."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0055","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1203\/01.PDR.0000136152.54638.95","article-title":"A galactosylceramide binding domain is involved in trafficking of CLN3 from Golgi to rafts via recycling endosomes","volume":"56","author":"Persaud-Sawin","year":"2004","journal-title":"Pediatr. Res."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0060","doi-asserted-by":"crossref","DOI":"10.3390\/ijms19020625","article-title":"Altered expression of ganglioside metabolizing enzymes results in GM3 ganglioside accumulation in cerebellar cells of a mouse model of juvenile neuronal ceroid lipofuscinosis","volume":"19","author":"Somogyi","year":"2018","journal-title":"Int. J. Mol. Sci."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0065","doi-asserted-by":"crossref","first-page":"249","DOI":"10.3389\/fnins.2018.00249","article-title":"Sphingolipid metabolism: a new therapeutic opportunity for brain degenerative disorders","volume":"12","author":"Di Pardo","year":"2018","journal-title":"Front. Neurosci."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0070","doi-asserted-by":"crossref","first-page":"5280","DOI":"10.1038\/s41598-017-05709-y","article-title":"Defective Sphingosine-1-phosphate metabolism is a druggable target in Huntington's disease","volume":"7","author":"Di Pardo","year":"2017","journal-title":"Sci. Rep."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0075","doi-asserted-by":"crossref","first-page":"1141","DOI":"10.1016\/j.bbrc.2016.08.082","article-title":"LRRK2 deficiency impacts ceramide metabolism in brain","volume":"478","author":"Ferrazza","year":"2016","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0080","doi-asserted-by":"crossref","first-page":"2070","DOI":"10.1073\/pnas.0305799101","article-title":"Involvement of oxidative stress-induced abnormalities in ceramide and cholesterol metabolism in brain aging and Alzheimer's disease","volume":"101","author":"Cutler","year":"2004","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0085","doi-asserted-by":"crossref","first-page":"12","DOI":"10.1186\/2051-5960-2-12","article-title":"Reduced sphingosine kinase-1 and enhanced sphingosine 1-phosphate lyase expression demonstrate deregulated sphingosine 1-phosphate signaling in Alzheimer's disease","volume":"2","author":"Ceccom","year":"2014","journal-title":"Acta Neuropathol Commun"},{"key":"10.1016\/j.bbadis.2018.11.011_bb0090","doi-asserted-by":"crossref","first-page":"260","DOI":"10.5114\/fn.2014.45567","article-title":"The key role of sphingosine kinases in the molecular mechanism of neuronal cell survival and death in an experimental model of Parkinson's disease","volume":"52","author":"Pyszko","year":"2014","journal-title":"Folia Neuropathol."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0095","doi-asserted-by":"crossref","first-page":"496","DOI":"10.1194\/jlr.M300347-JLR200","article-title":"The structural requirements for ceramide activation of serine-threonine protein phosphatases","volume":"45","author":"Chalfant","year":"2004","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0100","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1096\/fj.08-120550","article-title":"Direct interaction between the inhibitor 2 and ceramide via sphingolipid-protein binding is involved in the regulation of protein phosphatase 2A activity and signaling","volume":"23","author":"Mukhopadhyay","year":"2009","journal-title":"FASEB J."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0105","doi-asserted-by":"crossref","first-page":"911","DOI":"10.1139\/y59-099","article-title":"A rapid method of total lipid extraction and purification","volume":"37","author":"Bligh","year":"1959","journal-title":"Can. J. Biochem. Physiol."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0110","doi-asserted-by":"crossref","first-page":"36624","DOI":"10.1074\/jbc.M807377200","article-title":"ABCA12 maintains the epidermal lipid permeability barrier by facilitating formation of ceramide linoleic esters","volume":"283","author":"Zuo","year":"2008","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0115","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1194\/jlr.M600449-JLR200","article-title":"ABCA3 inactivation in mice causes respiratory failure, loss of pulmonary surfactant, and depletion of lung phosphatidylglycerol","volume":"48","author":"Fitzgerald","year":"2007","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0120","doi-asserted-by":"crossref","first-page":"186","DOI":"10.1016\/S0968-0004(99)01375-4","article-title":"Regulation of protein kinase cascades by protein phosphatase 2A","volume":"24","author":"Millward","year":"1999","journal-title":"Trends Biochem. Sci."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0125","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1146\/annurev.bi.58.070189.002321","article-title":"The structure and regulation of protein phosphatases","volume":"58","author":"Cohen","year":"1989","journal-title":"Annu. Rev. Biochem."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0130","doi-asserted-by":"crossref","first-page":"15523","DOI":"10.1016\/S0021-9258(18)82288-8","article-title":"Ceramide activates heterotrimeric protein phosphatase 2A","volume":"268","author":"Dobrowsky","year":"1993","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0135","doi-asserted-by":"crossref","first-page":"11867","DOI":"10.1073\/pnas.89.24.11867","article-title":"Cantharidin-binding protein: identification as protein phosphatase 2A","volume":"89","author":"Li","year":"1992","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0140","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1002\/humu.20676","article-title":"Gaucher disease: mutation and polymorphism spectrum in the glucocerebrosidase gene (GBA)","volume":"29","author":"Hruska","year":"2008","journal-title":"Hum. Mutat."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0145","doi-asserted-by":"crossref","first-page":"3748","DOI":"10.1073\/pnas.88.9.3748","article-title":"Niemann-pick disease: a frequent missense mutation in the acid sphingomyelinase gene of Ashkenazi Jewish type a and B patients","volume":"88","author":"Levran","year":"1991","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0150","first-page":"113","article-title":"Golgi staining by two fluorescent ceramide analogues in cultured fibroblasts requires metabolism","volume":"68","author":"Putz","year":"1995","journal-title":"Eur. J. Cell Biol."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0155","doi-asserted-by":"crossref","first-page":"377","DOI":"10.1002\/ajh.23118","article-title":"Genome-wide association study of N370S homozygous Gaucher disease reveals the candidacy of CLN8 gene as a genetic modifier contributing to extreme phenotypic variation","volume":"87","author":"Zhang","year":"2012","journal-title":"Am. J. Hematol."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0160","first-page":"123","article-title":"Both the establishment and the maintenance of neuronal polarity require active mechanisms: critical roles of GSK-3beta and its upstream regulators","volume":"120","author":"Jiang","year":"2005","journal-title":"Cell"},{"key":"10.1016\/j.bbadis.2018.11.011_bb0165","doi-asserted-by":"crossref","first-page":"ra15","DOI":"10.1126\/scisignal.2003661","article-title":"Akt and PP2A reciprocally regulate the guanine nucleotide exchange factor Dock6 to control axon growth of sensory neurons","volume":"6","author":"Miyamoto","year":"2013","journal-title":"Sci. Signal."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0170","doi-asserted-by":"crossref","first-page":"1750","DOI":"10.1007\/s12035-017-0444-3","article-title":"Protein phosphatase 2A: a double-faced phosphatase of cellular system and its role in neurodegenerative disorders","volume":"55","author":"Nematullah","year":"2018","journal-title":"Mol. Neurobiol."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0175","doi-asserted-by":"crossref","first-page":"16","DOI":"10.3389\/fnmol.2014.00016","article-title":"Protein phosphatase 2A dysfunction in Alzheimer's disease","volume":"7","author":"Sontag","year":"2014","journal-title":"Front. Mol. Neurosci."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0180","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1002\/acn3.337","article-title":"Dysregulation of protein phosphatase 2A in Parkinson disease and dementia with Lewy bodies","volume":"3","author":"Park","year":"2016","journal-title":"Ann Clin Transl Neurol"},{"key":"10.1016\/j.bbadis.2018.11.011_bb0185","doi-asserted-by":"crossref","first-page":"458","DOI":"10.1016\/j.ceb.2011.04.006","article-title":"Lipid transfer and signaling at organelle contact sites: the tip of the iceberg","volume":"23","author":"Toulmay","year":"2011","journal-title":"Curr. Opin. Cell Biol."},{"key":"10.1016\/j.bbadis.2018.11.011_bb0190","doi-asserted-by":"crossref","first-page":"529","DOI":"10.1016\/j.bbamcr.2012.10.030","article-title":"Identifying protein partners of CLN8, an ER-resident protein involved in neuronal ceroid lipofuscinosis","volume":"1833","author":"Passantino","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2018.11.011_bb0195","doi-asserted-by":"crossref","first-page":"952","DOI":"10.1016\/j.bbalip.2016.02.009","article-title":"VAP, a versatile access point for the endoplasmic reticulum: review and analysis of FFAT-like motifs in the VAPome","volume":"1861 (","author":"Murphy","year":"2016","journal-title":"Biochim. Biophys. Acta"}],"container-title":["Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0925443918304721?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0925443918304721?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,14]],"date-time":"2025-10-14T04:44:33Z","timestamp":1760417073000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0925443918304721"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,2]]},"references-count":39,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2019,2]]}},"alternative-id":["S0925443918304721"],"URL":"https:\/\/doi.org\/10.1016\/j.bbadis.2018.11.011","relation":{},"ISSN":["0925-4439"],"issn-type":[{"value":"0925-4439","type":"print"}],"subject":[],"published":{"date-parts":[[2019,2]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Neuronal ceroid lipofuscinosis related ER membrane protein CLN8 regulates PP2A activity and ceramide levels","name":"articletitle","label":"Article Title"},{"value":"Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.bbadis.2018.11.011","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2018 Elsevier B.V.","name":"copyright","label":"Copyright"}]}}