{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T02:14:24Z","timestamp":1789179264513,"version":"build-2803163510"},"reference-count":51,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2016,9,17]],"date-time":"2016-09-17T00:00:00Z","timestamp":1474070400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000065","name":"National Institute of Neurological Disorders and Stroke","doi-asserted-by":"crossref","award":["F32NS082005 (to T.S.-F.)"],"award-info":[{"award-number":["F32NS082005 (to T.S.-F.)"]}],"id":[{"id":"10.13039\/100000065","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Legacy for Angels Foundation","award":["R01NS065808 (to E.R.B.)"],"award-info":[{"award-number":["R01NS065808 (to E.R.B.)"]}]},{"name":"Legacy for Angels Foundation","award":["R21NS087474 (to E.R.B.)"],"award-info":[{"award-number":["R21NS087474 (to E.R.B.)"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Neuroscience Research"],"published-print":{"date-parts":[[2016,11]]},"abstract":"<jats:p>Sphingolipidoses are a class of inherited diseases that result from the toxic accumulation of undigested sphingolipids in lysosomes and other cellular membranes. Sphingolipids are particularly enriched in cells of the nervous system, and their excessive accumulation during disease has a significant impact on the nervous system. Neuronal dysfunction followed by neurological compromise is a common feature in many of these diseases; however, the underlying mechanisms that cause vulnerability of neurons are not fully understood. The plasma membrane plays a critical role in regulating cellular survival pathways, and its dysfunction has been implicated in neuronal failure in various adult\u2010onset neuropathies. In the context of sphingolipidoses, we hypothesize that gradual accumulation of undigested lipids in plasma membranes causes local disruptions in lipid raft domains, leading to deregulation of multiple signaling pathways important for neuronal survival and function. We propose that defects in downstream signaling as a result of membrane dysfunction are common mechanisms underlying neuronal vulnerability in sphingolipid storage disorders with neurological compromise. \u00a9 2016 Wiley Periodicals, Inc.<\/jats:p>","DOI":"10.1002\/jnr.23763","type":"journal-article","created":{"date-parts":[[2016,9,17]],"date-time":"2016-09-17T11:22:31Z","timestamp":1474111351000},"page":"1042-1048","source":"Crossref","is-referenced-by-count":18,"title":["How membrane dysfunction influences neuronal survival pathways in sphingolipid storage disorders"],"prefix":"10.1002","volume":"94","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8217-237X","authenticated-orcid":false,"given":"Tuba","family":"Sural\u2010Fehr","sequence":"first","affiliation":[{"name":"Department of Anatomy and Cell Biology University of Illinois at Chicago Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ernesto R.","family":"Bongarzone","sequence":"additional","affiliation":[{"name":"Department of Anatomy and Cell Biology University of Illinois at Chicago Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,9,17]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pneurobio.2014.02.005"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M201587200"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.23753"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.0217-13.2013"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.2431-14.2015"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-011-0814-2"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.170008497"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.10634"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/path.3021"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.23750"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.1692-04.2004"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02780608"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.2001.00132.x"},{"key":"e_1_2_11_15_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M115.664367"},{"key":"e_1_2_11_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2013.11.009"},{"key":"e_1_2_11_17_1","doi-asserted-by":"publisher","DOI":"10.1194\/jlr.M039610"},{"key":"e_1_2_11_18_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.23-08-03262.2003"},{"key":"e_1_2_11_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cub.2007.06.062"},{"key":"e_1_2_11_20_1","doi-asserted-by":"publisher","DOI":"10.1146\/annurev.neuro.24.1.677"},{"key":"e_1_2_11_21_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M211785200"},{"key":"e_1_2_11_22_1","doi-asserted-by":"publisher","DOI":"10.1038\/73888"},{"key":"e_1_2_11_23_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.expneurol.2012.06.003"},{"key":"e_1_2_11_24_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2013.10.010"},{"key":"e_1_2_11_25_1","doi-asserted-by":"publisher","DOI":"10.1210\/jc.2007-1702"},{"key":"e_1_2_11_26_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.23-08-03164.2003"},{"key":"e_1_2_11_27_1","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/21.3.281"},{"key":"e_1_2_11_28_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0065235"},{"key":"e_1_2_11_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ab.2014.08.033"},{"key":"e_1_2_11_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tins.2005.07.003"},{"key":"e_1_2_11_31_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M403816200"},{"key":"e_1_2_11_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0959-4388(02)00363-X"},{"key":"e_1_2_11_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0896-6273(01)00188-X"},{"key":"e_1_2_11_34_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.272.1.154"},{"key":"e_1_2_11_35_1","doi-asserted-by":"publisher","DOI":"10.1194\/jlr.R200021-JLR200"},{"key":"e_1_2_11_36_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M115.636498"},{"key":"e_1_2_11_37_1","doi-asserted-by":"publisher","DOI":"10.1098\/rstb.2006.1894"},{"key":"e_1_2_11_38_1","doi-asserted-by":"publisher","DOI":"10.1002\/jnr.22099"},{"key":"e_1_2_11_39_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-004-0952-x"},{"key":"e_1_2_11_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuron.2008.01.027"},{"key":"e_1_2_11_41_1","doi-asserted-by":"publisher","DOI":"10.1038\/42408"},{"key":"e_1_2_11_42_1","doi-asserted-by":"publisher","DOI":"10.1128\/MCB.19.11.7289"},{"key":"e_1_2_11_43_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.200404106"},{"key":"e_1_2_11_44_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neuropharm.2009.05.013"},{"key":"e_1_2_11_45_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nbd.2014.02.012"},{"key":"e_1_2_11_46_1","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20050460"},{"key":"e_1_2_11_47_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.5597-08.2009"},{"key":"e_1_2_11_48_1","doi-asserted-by":"publisher","DOI":"10.1002\/dneu.20765"},{"key":"e_1_2_11_49_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mcn.2005.08.004"},{"issue":"1","key":"e_1_2_11_50_1","first-page":"11","article-title":"Trafficking and functions of bioactive sphingolipids: lessons from cells and model membranes","volume":"8","author":"Zhou K","year":"2015","journal-title":"Lipid Insights"},{"key":"e_1_2_11_51_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1471-4159.1995.65052241.x"},{"key":"e_1_2_11_52_1","doi-asserted-by":"publisher","DOI":"10.3389\/fnsyn.2013.00009"}],"container-title":["Journal of Neuroscience Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fjnr.23763","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/jnr.23763","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,13]],"date-time":"2023-09-13T15:11:38Z","timestamp":1694617898000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/jnr.23763"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,9,17]]},"references-count":51,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2016,11]]}},"alternative-id":["10.1002\/jnr.23763"],"URL":"https:\/\/doi.org\/10.1002\/jnr.23763","archive":["Portico"],"relation":{},"ISSN":["0360-4012","1097-4547"],"issn-type":[{"value":"0360-4012","type":"print"},{"value":"1097-4547","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,9,17]]}}}