{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,2]],"date-time":"2026-06-02T13:09:58Z","timestamp":1780405798083,"version":"3.54.1"},"reference-count":53,"publisher":"Wiley","issue":"18","license":[{"start":{"date-parts":[[2019,8,29]],"date-time":"2019-08-29T00:00:00Z","timestamp":1567036800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100008522","name":"University of Illinois at Chicago","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008522","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100008109","name":"University of Notre Dame","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100008109","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["analyticalsciencejournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Proteomics"],"published-print":{"date-parts":[[2019,9]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Niemann\u2013Pick disease, type C1 (NPC1) is a fatal, autosomal recessive, neurodegenerative disorder caused by mutations in the\n                    <jats:italic>NPC1<\/jats:italic>\n                    gene. As a result of the genetic defect, there is accumulation of unesterified cholesterol and sphingolipids in the late endosomal\/lysosomal system causing both visceral and neurological defects. These manifest clinically as hepatosplenomegaly, liver dysfunction, and neurodegeneration. While significant progress has been made to better understand NPC1, the downstream effects of cholesterol storage and the major mechanisms that drive these pathologies remains less understood. In this study, it is sought to investigate free fatty acid levels in\n                    <jats:italic>\n                      Npc1\n                      <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    <\/jats:italic>\n                    mice with focus on the polyunsaturated \u03c9\u20103 and \u03c9\u20106 fatty acids. Since fatty acids are the main constituents of numerous lipids species, a discovery based lipidomic study of liver tissue in Npc\n                    <jats:italic>\n                      1\n                      <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    <\/jats:italic>\n                    mice is also performed. To this end, alterations in fatty acid synthesis, including the \u03c9\u20103 and 6 fatty acids, are reported. Further, alterations in enzymes that regulate the synthesis of \u03c9\u20103 and 6 fatty acids are reported. Analysis of the liver lipidome reveals alterations in both storage and membrane lipids including ceramides, fatty acids, phosphatidylcholamines, phosphatidylglycerols, phosphatidylethanolamines, sphingomyelins, and triacylglycerols in\n                    <jats:italic>\n                      Npc1\n                      <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    <\/jats:italic>\n                    mice at a late stage of disease.\n                  <\/jats:p>","DOI":"10.1002\/pmic.201800285","type":"journal-article","created":{"date-parts":[[2019,8,8]],"date-time":"2019-08-08T19:33:45Z","timestamp":1565292825000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Lipidomic Analysis Reveals Altered Fatty Acid Metabolism in the Liver of the Symptomatic Niemann\u2013Pick, Type C1 Mouse Model"],"prefix":"10.1002","volume":"19","author":[{"given":"Melissa R.","family":"Pergande","sequence":"first","affiliation":[{"name":"Department of Chemistry University of Illinois at Chicago  Chicago IL 60607 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fidel","family":"Serna\u2010Perez","sequence":"additional","affiliation":[{"name":"Department of Chemistry University of Illinois at Chicago  Chicago IL 60607 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sheher Banu","family":"Mohsin","sequence":"additional","affiliation":[{"name":"Agilent Technologies  Wood Dale IL 60191 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathon","family":"Hanek","sequence":"additional","affiliation":[{"name":"Department of Chemistry University of Illinois at Chicago  Chicago IL 60607 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3541-3361","authenticated-orcid":false,"given":"Stephanie M.","family":"Cologna","sequence":"additional","affiliation":[{"name":"Department of Chemistry University of Illinois at Chicago  Chicago IL 60607 USA"},{"name":"Department of Chemistry Laboratory for Integrative Neuroscience University of Illinois at Chicago  Chicago IL 60607 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,8,29]]},"reference":[{"key":"e_1_2_8_1_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1399-0004.2003.00147.x"},{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbalip.2004.08.014"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1388-1981(00)00158-X"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(05)81695-6"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1194\/jlr.M700525-JLR200"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2011.08.096"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.20868"},{"key":"e_1_2_8_8_1","first-page":"140","volume":"108","author":"Morris M. 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