{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,17]],"date-time":"2026-01-17T19:25:37Z","timestamp":1768677937767,"version":"3.49.0"},"reference-count":49,"publisher":"Elsevier BV","issue":"8","license":[{"start":{"date-parts":[[2018,11,1]],"date-time":"2018-11-01T00:00:00Z","timestamp":1541030400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2018,11,1]],"date-time":"2018-11-01T00:00:00Z","timestamp":1541030400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/501100004359","name":"Swedish Research Council","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100004359","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100009673","name":"Gun and Bertil Stohne\u2019s Foundation","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100009673","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Olle Engkvist Byggm\u00e4stare Foundation"},{"DOI":"10.13039\/100010815","name":"Foundation for Old Servants","doi-asserted-by":"crossref","id":[{"id":"10.13039\/100010815","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/100010773","name":"Dementia Association","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010773","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Karolinska Institutet Foundations"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["European Journal of Cell Biology"],"published-print":{"date-parts":[[2018,11]]},"DOI":"10.1016\/j.ejcb.2018.10.001","type":"journal-article","created":{"date-parts":[[2018,10,22]],"date-time":"2018-10-22T02:52:08Z","timestamp":1540176728000},"page":"557-567","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":25,"title":["Autophagy-lysosomal defect in human CADASIL vascular smooth muscle cells"],"prefix":"10.1016","volume":"97","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0795-8348","authenticated-orcid":false,"given":"Evelyn S.","family":"Hanemaaijer","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mahmod","family":"Panahi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nol","family":"Swaddiwudhipong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Saara","family":"Tikka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bengt","family":"Winblad","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matti","family":"Viitanen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio","family":"Piras","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Homira","family":"Behbahani","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.ejcb.2018.10.001_bib0005","first-page":"36","article-title":"Image processing with ImageJ","volume":"11","author":"Abramoff","year":"2004","journal-title":"Biophoton. Int."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0010","doi-asserted-by":"crossref","first-page":"3593","DOI":"10.1242\/dev.063610","article-title":"Notch signaling: simplicity in design, versatility in function","volume":"138","author":"Andersson","year":"2011","journal-title":"Development"},{"key":"10.1016\/j.ejcb.2018.10.001_bib0015","doi-asserted-by":"crossref","first-page":"E128","DOI":"10.1073\/pnas.1101964108","article-title":"Hypomorphic Notch 3 alleles link Notch signaling to ischemic cerebral small-vessel disease","volume":"108","author":"Arboleda-Velasquez","year":"2011","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0020","doi-asserted-by":"crossref","first-page":"770","DOI":"10.1126\/science.284.5415.770","article-title":"Notch signaling: cell fate control and signal integration in development","volume":"284","author":"Artavanis-Tsakonas","year":"1999","journal-title":"Science"},{"key":"10.1016\/j.ejcb.2018.10.001_bib0025","doi-asserted-by":"crossref","first-page":"192","DOI":"10.1111\/j.1471-4159.2005.03181.x","article-title":"Sequestosome 1\/p62 shuttles polyubiquitinated tau for proteasomal degradation","volume":"94","author":"Babu","year":"2005","journal-title":"J. Neurochem."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0030","doi-asserted-by":"crossref","first-page":"2594","DOI":"10.1016\/j.lfs.2005.04.020","article-title":"Modifications in P62 occur due to proteasome inhibition in alcoholic liver disease","volume":"77","author":"Bardag-Gorce","year":"2005","journal-title":"Life Sci."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0035","doi-asserted-by":"crossref","first-page":"138","DOI":"10.4161\/auto.2.2.2405","article-title":"p62\/SQSTM1: a missing link between protein aggregates and the autophagy machinery","volume":"2","author":"Bjorkoy","year":"2006","journal-title":"Autophagy"},{"key":"10.1016\/j.ejcb.2018.10.001_bib0040","doi-asserted-by":"crossref","first-page":"1631","DOI":"10.1007\/s00018-009-8668-7","article-title":"The Notch signaling pathway: transcriptional regulation at Notch target genes","volume":"66","author":"Borggrefe","year":"2009","journal-title":"Cell. Mol. Life Sci."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0045","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1038\/nrm1552","article-title":"Proteolysis: from the lysosome to ubiquitin and the proteasome","volume":"6","author":"Ciechanover","year":"2005","journal-title":"Nat. Rev. Mol. Cell. Biol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0050","doi-asserted-by":"crossref","first-page":"e147","DOI":"10.1038\/emm.2014.117","article-title":"Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies","volume":"47","author":"Ciechanover","year":"2015","journal-title":"Exp. Mol. Med."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0055","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1002\/ana.410440506","article-title":"The phenotypic spectrum of CADASIL: clinical findings in 102 cases","volume":"44","author":"Dichgans","year":"1998","journal-title":"Ann. Neurol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0060","first-page":"473","article-title":"Chloroquine-induced autophagic vacuole accumulation and cell death in glioma cells is p53 independent","volume":"12","author":"Geng","year":"2010","journal-title":"Neuro-Oncology"},{"key":"10.1016\/j.ejcb.2018.10.001_bib0065","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1083\/jcb.60.3.673","article-title":"Human vascular endothelial cells in culture. Growth and DNA synthesis","volume":"60","author":"Gimbrone","year":"1974","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0070","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1097\/nen.0b013e318093e574","article-title":"Apoptosis in cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy","volume":"66","author":"Gray","year":"2007","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0075","doi-asserted-by":"crossref","first-page":"48","DOI":"10.3109\/01913123.2011.620220","article-title":"Ultrastructural changes in cerebral capillary pericytes in aged Notch3 mutant transgenic mice","volume":"36","author":"Gu","year":"2012","journal-title":"Ultrastruct. Pathol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0080","doi-asserted-by":"crossref","DOI":"10.1111\/bpa.12621","article-title":"Severe white matter astrocytopathy in CADASIL","author":"Hase","year":"2018","journal-title":"Brain Pathol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0085","doi-asserted-by":"crossref","first-page":"305","DOI":"10.2119\/2006-00069.Ihalainen","article-title":"Proteome analysis of cultivated vascular smooth muscle cells from a CADASIL patient","volume":"13","author":"Ihalainen","year":"2007","journal-title":"Mol. Med."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0090","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1007\/s00401-006-0116-2","article-title":"Notch3 ectodomain is a major component of granular osmiophilic material (GOM) in CADASIL","volume":"112","author":"Ishiko","year":"2006","journal-title":"Acta Neuropathol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0095","doi-asserted-by":"crossref","first-page":"2594","DOI":"10.1016\/j.biocel.2009.08.019","article-title":"Lysosome-dependent degradation of Notch3","volume":"41","author":"Jia","year":"2009","journal-title":"Int. J. Biochem. Cell Biol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0100","doi-asserted-by":"crossref","first-page":"707","DOI":"10.1038\/383707a0","article-title":"Notch3 mutations in CADASIL, a hereditary adult-onset condition causing stroke and dementia","volume":"383","author":"Joutel","year":"1996","journal-title":"Nature"},{"key":"10.1016\/j.ejcb.2018.10.001_bib0105","doi-asserted-by":"crossref","first-page":"1511","DOI":"10.1016\/S0140-6736(97)08083-5","article-title":"Strong clustering and stereotyped nature of Notch3 mutations in CADASIL patients","volume":"350","author":"Joutel","year":"1997","journal-title":"Lancet"},{"key":"10.1016\/j.ejcb.2018.10.001_bib0110","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1172\/JCI8047","article-title":"The ectodomain of the Notch3 receptor accumulates within the cerebrovasculature of CADASIL patients","volume":"105","author":"Joutel","year":"2000","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0115","doi-asserted-by":"crossref","first-page":"17119","DOI":"10.1073\/pnas.252624099","article-title":"A CADASIL-mutated Notch 3 receptor exhibits impaired intracellular trafficking and maturation but normal ligand-induced signaling","volume":"99","author":"Karlstrom","year":"2002","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0120","doi-asserted-by":"crossref","first-page":"445","DOI":"10.4161\/auto.19496","article-title":"Guidelines for the use and interpretation of assays for monitoring autophagy","volume":"8","author":"Klionsky","year":"2012","journal-title":"Autophagy"},{"key":"10.1016\/j.ejcb.2018.10.001_bib0125","doi-asserted-by":"crossref","first-page":"223","DOI":"10.1006\/bbrc.2000.4107","article-title":"Ubiquitin-binding protein p62 expression is induced during apoptosis and proteasomal inhibition in neuronal cells","volume":"280","author":"Kuusisto","year":"2001","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0130","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1042\/BJ20110892","article-title":"Regulation and function of ribosomal protein S6 kinase (S6K) within mTOR signalling networks","volume":"441","author":"Magnuson","year":"2012","journal-title":"Biochem. J."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0135","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1016\/j.tins.2016.02.002","article-title":"The autophagy-lysosomal pathway in neurodegeneration: a TFEB perspective","volume":"39","author":"Martini-Stoica","year":"2016","journal-title":"Trends Neurosci."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0140","doi-asserted-by":"crossref","first-page":"1435","DOI":"10.1080\/15548627.2018.1474314","article-title":"Chloroquine inhibits autophagic flux by decreasing autophagosome-lysosome fusion","volume":"14","author":"Mauthe","year":"2018","journal-title":"Autophagy"},{"key":"10.1016\/j.ejcb.2018.10.001_bib0145","doi-asserted-by":"crossref","first-page":"358","DOI":"10.1111\/j.1750-3639.2004.tb00078.x","article-title":"Fibrosis and stenosis of the long penetrating cerebral arteries: the cause of the white matter pathology in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy","volume":"14","author":"Miao","year":"2004","journal-title":"Brain Pathol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0150","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1016\/j.brainres.2004.03.029","article-title":"Transcriptional activation of p62\/A170\/ZIP during the formation of the aggregates: possible mechanisms and the role in Lewy body formation in Parkinson\u2019s disease","volume":"1012","author":"Nakaso","year":"2004","journal-title":"Brain Res."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0155","doi-asserted-by":"crossref","first-page":"2761","DOI":"10.1093\/hmg\/ddp211","article-title":"CADASIL mutations enhance spontaneous multimerization of NOTCH3","volume":"18","author":"Opherk","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0160","doi-asserted-by":"crossref","first-page":"3016","DOI":"10.1111\/jcmm.13534","article-title":"Differences in proliferation rate between CADASIL and control vascular smooth muscle cells are related to increased TGFbeta expression","volume":"22","author":"Panahi","year":"2018","journal-title":"J. Cell. Mol. Med."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0165","doi-asserted-by":"crossref","first-page":"24131","DOI":"10.1074\/jbc.M702824200","article-title":"p62\/SQSTM1 binds directly to Atg8\/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy","volume":"282","author":"Pankiv","year":"2007","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0170","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1006\/excr.2002.5544","article-title":"Mouse Notch 3 expression in the pre- and postnatal brain: relationship to the stroke and dementia syndrome CADASIL","volume":"278","author":"Prakash","year":"2002","journal-title":"Exp. Cell Res."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0175","doi-asserted-by":"crossref","first-page":"585","DOI":"10.4161\/auto.5.5.8823","article-title":"In search of an \"autophagomometer\"","volume":"5","author":"Rubinsztein","year":"2009","journal-title":"Autophagy"},{"key":"10.1016\/j.ejcb.2018.10.001_bib0180","doi-asserted-by":"crossref","first-page":"947","DOI":"10.1097\/00005072-199709000-00001","article-title":"CADASIL: cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy","volume":"56","author":"Ruchoux","year":"1997","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0185","doi-asserted-by":"crossref","first-page":"2291","DOI":"10.1161\/01.STR.25.11.2291","article-title":"Presence of ultrastructural arterial lesions in muscle and skin vessels of patients with CADASIL","volume":"25","author":"Ruchoux","year":"1994","journal-title":"Stroke"},{"key":"10.1016\/j.ejcb.2018.10.001_bib0190","doi-asserted-by":"crossref","first-page":"8055","DOI":"10.1128\/MCB.24.18.8055-8068.2004","article-title":"Sequestosome 1\/p62 is a polyubiquitin chain binding protein involved in ubiquitin proteasome degradation","volume":"24","author":"Seibenhener","year":"2004","journal-title":"Mol. Cell. Biol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0195","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.ijcard.2016.02.128","article-title":"The roles of autophagy in vascular smooth muscle cells","volume":"211","author":"Tai","year":"2016","journal-title":"Int. J. Cardiol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0200","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1093\/hmg\/ddp468","article-title":"Mutations in NOTCH3 cause the formation and retention of aggregates in the endoplasmic reticulum, leading to impaired cell proliferation","volume":"19","author":"Takahashi","year":"2010","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0205","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1002\/path.2094","article-title":"Autophagy, organelles and ageing","volume":"211","author":"Terman","year":"2007","journal-title":"J. Pathol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0210","doi-asserted-by":"crossref","first-page":"2171","DOI":"10.1038\/jcbfm.2012.123","article-title":"CADASIL mutations and shRNA silencing of NOTCH3 affect actin organization in cultured vascular smooth muscle cells","volume":"32","author":"Tikka","year":"2012","journal-title":"J. Cereb. Blood Flow Metab."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0215","doi-asserted-by":"crossref","first-page":"525","DOI":"10.1111\/bpa.12181","article-title":"CADASIL and CARASIL","volume":"24","author":"Tikka","year":"2014","journal-title":"Brain Pathol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0220","doi-asserted-by":"crossref","first-page":"134","DOI":"10.1016\/j.yexcr.2012.09.015","article-title":"Experimental studies of mitochondrial function in CADASIL vascular smooth muscle cells","volume":"319","author":"Viitanen","year":"2013","journal-title":"Exp. Cell Res."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0225","doi-asserted-by":"crossref","first-page":"21723","DOI":"10.1074\/jbc.M202224200","article-title":"Notch3 signaling in vascular smooth muscle cells induces c-FLIP expression via ERK\/MAPK activation. Resistance to Fas ligand-induced apoptosis","volume":"277","author":"Wang","year":"2002","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0230","doi-asserted-by":"crossref","first-page":"21412","DOI":"10.1074\/jbc.M109.026013","article-title":"A non-canonical MEK\/ERK signaling pathway regulates autophagy via regulating Beclin 1","volume":"284","author":"Wang","year":"2009","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0235","doi-asserted-by":"crossref","first-page":"430","DOI":"10.1083\/jcb.63.2.430","article-title":"Protein degradation in cultured cells. II. The uptake of chloroquine by rat fibroblasts and the inhibition of cellular protein degradation and cathepsin B1","volume":"63","author":"Wibo","year":"1974","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.ejcb.2018.10.001_bib0240","doi-asserted-by":"crossref","first-page":"437","DOI":"10.1093\/abbs\/gmp031","article-title":"The lysosome and neurodegenerative diseases","volume":"41","author":"Zhang","year":"2009","journal-title":"Acta Biochim. Biophys. Sin. (Shanghai)"},{"key":"10.1016\/j.ejcb.2018.10.001_bib0245","doi-asserted-by":"crossref","DOI":"10.1042\/BSR20140141","article-title":"MAPK\/JNK signalling: a potential autophagy regulation pathway","volume":"35","author":"Zhou","year":"2015","journal-title":"Biosci. Rep."}],"container-title":["European Journal of Cell Biology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0171933518300815?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0171933518300815?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,9,30]],"date-time":"2025-09-30T17:18:13Z","timestamp":1759252693000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0171933518300815"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,11]]},"references-count":49,"journal-issue":{"issue":"8","published-print":{"date-parts":[[2018,11]]}},"alternative-id":["S0171933518300815"],"URL":"https:\/\/doi.org\/10.1016\/j.ejcb.2018.10.001","relation":{},"ISSN":["0171-9335"],"issn-type":[{"value":"0171-9335","type":"print"}],"subject":[],"published":{"date-parts":[[2018,11]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Autophagy-lysosomal defect in human CADASIL vascular smooth muscle cells","name":"articletitle","label":"Article Title"},{"value":"European Journal of Cell Biology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ejcb.2018.10.001","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2018 Elsevier GmbH. All rights reserved.","name":"copyright","label":"Copyright"}]}}