{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,24]],"date-time":"2026-08-24T14:29:31Z","timestamp":1787581771734,"version":"build-2736575974"},"reference-count":71,"publisher":"MDPI AG","issue":"5","license":[{"start":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T00:00:00Z","timestamp":1621296000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft","doi-asserted-by":"publisher","award":["TU492\/3-1"],"award-info":[{"award-number":["TU492\/3-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Ultragenix","award":["UX007-NCIST217"],"award-info":[{"award-number":["UX007-NCIST217"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Long-chain fatty acid oxidation disorders (lc-FAOD) are a group of diseases affecting the degradation of long-chain fatty acids. In order to investigate the disease specific alterations of the cellular lipidome, we performed undirected lipidomics in fibroblasts from patients with carnitine palmitoyltransferase II, very long-chain acyl-CoA dehydrogenase, and long-chain 3-hydroxyacyl-CoA dehydrogenase. We demonstrate a deep remodeling of mitochondrial cardiolipins. The aberrant phosphatidylcholine\/phosphatidylethanolamine ratio and the increased content of plasmalogens and of lysophospholipids support the theory of an inflammatory phenotype in lc-FAOD. Moreover, we describe increased ratios of sphingomyelin\/ceramide and sphingomyelin\/hexosylceramide in LCHAD deficiency which may contribute to the neuropathic phenotype of LCHADD\/mitochondrial trifunctional protein deficiency.<\/jats:p>","DOI":"10.3390\/cells10051239","type":"journal-article","created":{"date-parts":[[2021,5,18]],"date-time":"2021-05-18T12:17:16Z","timestamp":1621340236000},"page":"1239","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Different Lipid Signature in Fibroblasts of Long-Chain Fatty Acid Oxidation Disorders"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2124-0092","authenticated-orcid":false,"given":"Khaled I.","family":"Alatibi","sequence":"first","affiliation":[{"name":"Department of General Pediatrics, Adolescent Medicine and Neonatology, Faculty of Medicine, Medical Centre-University of Freiburg, 79106 Freiburg, Germany"},{"name":"Faculty of Biology, University of Freiburg, 79104 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1396-3407","authenticated-orcid":false,"given":"Judith","family":"Hagenbuchner","sequence":"additional","affiliation":[{"name":"Department of Pediatrics II, Medical University Innsbruck, 6020 Innsbruck, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7253-2765","authenticated-orcid":false,"given":"Zeinab","family":"Wehbe","sequence":"additional","affiliation":[{"name":"Department of General Pediatrics, Adolescent Medicine and Neonatology, Faculty of Medicine, Medical Centre-University of Freiburg, 79106 Freiburg, Germany"},{"name":"Department of Pediatric Hematology and Oncology, Faculty of Medicine, Center of Pediatric and Adolescent Medicine-Medical Center-University of Freiburg, 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4846-1047","authenticated-orcid":false,"given":"Daniela","family":"Karall","sequence":"additional","affiliation":[{"name":"Department of Pediatrics I, Medical University Innsbruck, 6020 Innsbruck, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1015-2302","authenticated-orcid":false,"given":"Michael J.","family":"Ausserlechner","sequence":"additional","affiliation":[{"name":"Department of Pediatrics I, Medical University Innsbruck, 6020 Innsbruck, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8180-6457","authenticated-orcid":false,"given":"Jerry","family":"Vockley","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Pittsburgh, Pittsburgh, PA 15260, USA"},{"name":"Center for Rare Disease Therapy, UPMC Children\u2019s Hospital of Pittsburgh, Pittsburgh, PA 15224, USA"},{"name":"Graduate School of Public Health, University of Pittsburgh, Pittsburgh, PA 15260, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ute","family":"Spiekerkoetter","sequence":"additional","affiliation":[{"name":"Department of General Pediatrics, Adolescent Medicine and Neonatology, Faculty of Medicine, Medical Centre-University of Freiburg, 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5986-0468","authenticated-orcid":false,"given":"Sarah C.","family":"Gr\u00fcnert","sequence":"additional","affiliation":[{"name":"Department of General Pediatrics, Adolescent Medicine and Neonatology, Faculty of Medicine, Medical Centre-University of Freiburg, 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3851-4843","authenticated-orcid":false,"given":"Sara","family":"Tucci","sequence":"additional","affiliation":[{"name":"Department of General Pediatrics, Adolescent Medicine and Neonatology, Faculty of Medicine, Medical Centre-University of Freiburg, 79106 Freiburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,5,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1007\/s10545-010-9090-x","article-title":"Mitochondrial fatty acid oxidation disorders: Clinical presentation of long-chain fatty acid oxidation defects before and after newborn screening","volume":"33","author":"Spiekerkoetter","year":"2010","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"331","DOI":"10.1016\/0003-2697(89)90441-7","article-title":"Application of fast atom bombardment with tandem mass spectrometry and liquid chromatography\/mass spectrometry to the analysis of acylcarnitines in human urine, blood, and tissue","volume":"180","author":"Millington","year":"1989","journal-title":"Anal. Biochem."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"324","DOI":"10.1203\/00006450-199509000-00009","article-title":"Diagnosis of inborn errors of metabolism from blood spots by acylcarnitines and amino acids profiling using automated electrospray tandem mass spectrometry","volume":"38","author":"Rashed","year":"1995","journal-title":"Pediatr. Res."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1136\/jnnp.62.2.169","article-title":"Characterisation of carnitine palmitoyltransferases in patients with a carnitine palmitoyltransferase deficiency: Implications for diagnosis and therapy","volume":"62","author":"Schaefer","year":"1997","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1007\/s10545-010-9076-8","article-title":"Newborn screening for disorders of fatty-acid oxidation: Experience and recommendations from an expert meeting","volume":"33","author":"Lindner","year":"2010","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1016\/j.ymgme.2008.09.008","article-title":"A delphi clinical practice protocol for the management of very long chain acyl-coa dehydrogenase deficiency","volume":"96","author":"Arnold","year":"2009","journal-title":"Mol. Genet. Metab."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1007\/s10545-009-1126-8","article-title":"Treatment recommendations in long-chain fatty acid oxidation defects: Consensus from a workshop","volume":"32","author":"Spiekerkoetter","year":"2009","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"29834","DOI":"10.1074\/jbc.M110.139493","article-title":"Evidence for physical association of mitochondrial fatty acid oxidation and oxidative phosphorylation complexes","volume":"285","author":"Wang","year":"2010","journal-title":"J. Biol. Chem."},{"key":"ref_9","doi-asserted-by":"crossref","unstructured":"Ahmadpour, S.T., Maheo, K., Servais, S., Brisson, L., and Dumas, J.F. (2020). Cardiolipin, the mitochondrial signature lipid: Implication in cancer. Int. J. Mol. Sci., 21.","DOI":"10.3390\/ijms21218031"},{"key":"ref_10","doi-asserted-by":"crossref","unstructured":"Taylor, W.A., Mejia, E.M., Mitchell, R.W., Choy, P.C., Sparagna, G.C., and Hatch, G.M. (2012). Human trifunctional protein alpha links cardiolipin remodeling to beta-oxidation. PLoS ONE, 7.","DOI":"10.1371\/journal.pone.0048628"},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Zhang, Y., Bharathi, S.S., Rardin, M.J., Uppala, R., Verdin, E., Gibson, B.W., and Goetzman, E.S. (2015). Sirt3 and sirt5 regulate the enzyme activity and cardiolipin binding of very long-chain acyl-coa dehydrogenase. PLoS ONE, 10.","DOI":"10.1371\/journal.pone.0122297"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"3511","DOI":"10.1111\/febs.15221","article-title":"Sex-specific perturbation of complex lipids in response to medium-chain fatty acids in very long-chain acyl-coa dehydrogenase deficiency","volume":"287","author":"Alatibi","year":"2020","journal-title":"FEBS J."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"399","DOI":"10.1007\/s10545-016-9915-3","article-title":"Unique plasma metabolomic signatures of individuals with inherited disorders of long-chain fatty acid oxidation","volume":"39","author":"McCoin","year":"2016","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_14","doi-asserted-by":"crossref","unstructured":"Sklirou, E., Alodaib, A.N., Dobrowolski, S.F., Mohsen, A.-W.A., and Vockley, J. (2021). Physiological perspectives on the use of triheptanoin as anaplerotic therapy for long chain fatty acid oxidation disorders. Front. Genet., 11.","DOI":"10.3389\/fgene.2020.598760"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"3254","DOI":"10.1038\/s41598-018-21519-2","article-title":"Very long-\/and long chain-3-hydroxy acyl coa dehydrogenase deficiency correlates with deregulation of the mitochondrial fusion\/fission machinery","volume":"8","author":"Hagenbuchner","year":"2018","journal-title":"Sci. Rep."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1169","DOI":"10.1007\/s10545-018-0245-5","article-title":"The diagnostic challenge in very-long chain acyl-coa dehydrogenase deficiency (vlcadd)","volume":"41","author":"Hesse","year":"2018","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"1903","DOI":"10.1073\/pnas.1019267108","article-title":"Membrane lipidome of an epithelial cell line","volume":"108","author":"Sampaio","year":"2011","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"2136","DOI":"10.1073\/pnas.0811700106","article-title":"Global analysis of the yeast lipidome by quantitative shotgun mass spectrometry","volume":"106","author":"Ejsing","year":"2009","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1540","DOI":"10.1002\/ejlt.201500145","article-title":"An automated shotgun lipidomics platform for high throughput, comprehensive, and quantitative analysis of blood plasma intact lipids","volume":"117","author":"Surma","year":"2015","journal-title":"Eur. J. Lipid Sci. Technol. EJLST"},{"key":"ref_20","doi-asserted-by":"crossref","unstructured":"Herzog, R., Schuhmann, K., Schwudke, D., Sampaio, J.L., Bornstein, S.R., Schroeder, M., and Shevchenko, A. (2012). Lipidxplorer: A software for consensual cross-platform lipidomics. PLoS ONE, 7.","DOI":"10.1371\/journal.pone.0029851"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"R8","DOI":"10.1186\/gb-2011-12-1-r8","article-title":"A novel informatics concept for high-throughput shotgun lipidomics based on the molecular fragmentation query language","volume":"12","author":"Herzog","year":"2011","journal-title":"Genome Biol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"619","DOI":"10.1124\/jpet.112.192138","article-title":"Mitochondrial dysfunction in neurodegenerative diseases","volume":"342","author":"Johri","year":"2012","journal-title":"J. Pharmacol. Exp. Ther."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"993","DOI":"10.1007\/s00109-010-0663-9","article-title":"Mitochondrial biogenesis in the metabolic syndrome and cardiovascular disease","volume":"88","author":"Ren","year":"2010","journal-title":"J. Mol. Med."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1016\/j.tem.2012.06.002","article-title":"Mitochondrial dysfunction in pancreatic beta cells","volume":"23","author":"Supale","year":"2012","journal-title":"Trends Endocrinol. Metab."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1558","DOI":"10.1016\/j.bbamem.2017.04.006","article-title":"The critical role of phosphatidylcholine and phosphatidylethanolamine metabolism in health and disease","volume":"1859","author":"Kennelly","year":"2017","journal-title":"Biochim. Biophys. Acta Biomembr."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1591","DOI":"10.1016\/j.bbalip.2019.08.001","article-title":"The fate of medium-chain fatty acids in very long-chain acylcoa dehydrogenase deficiency (vlcadd): A matter of sex?","volume":"1864","author":"Wehbe","year":"2019","journal-title":"Biochim. Biophys. Acta Mol. Cell Biol. Lipids"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"4671","DOI":"10.1038\/s41467-019-12482-1","article-title":"Tfpa\/hadha is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes","volume":"10","author":"Miklas","year":"2019","journal-title":"Nat. Commun."},{"key":"ref_28","doi-asserted-by":"crossref","unstructured":"Wasmus, C., and Dudek, J. (2020). Metabolic alterations caused by defective cardiolipin remodeling in inherited cardiomyopathies. Life, 10.","DOI":"10.3390\/life10110277"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"12380","DOI":"10.1074\/jbc.RA119.008680","article-title":"Mitochondrial fatty acid oxidation and the electron transport chain comprise a multifunctional mitochondrial protein complex","volume":"294","author":"Wang","year":"2019","journal-title":"J. Biol. Chem."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1038\/nrm.2017.107","article-title":"Sphingolipids and their metabolism in physiology and disease","volume":"19","author":"Hannun","year":"2018","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"ref_31","doi-asserted-by":"crossref","unstructured":"Jatooratthawichot, P., Talabnin, C., Ngiwsara, L., Rustam, Y.H., Svasti, J., Reid, G.E., and Ketudat Cairns, J.R. (2020). Effect of expression of human glucosylceramidase 2 isoforms on lipid profiles in cos-7 cells. Metabolites, 10.","DOI":"10.1101\/2020.07.06.190314"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1007\/s11154-018-9448-1","article-title":"Disorders of mitochondrial long-chain fatty acid oxidation and the carnitine shuttle","volume":"19","author":"Knottnerus","year":"2018","journal-title":"Rev. Endocr. Metab. Disord."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"528","DOI":"10.1038\/nature09968","article-title":"Aberrant lipid metabolism disrupts calcium homeostasis causing liver endoplasmic reticulum stress in obesity","volume":"473","author":"Fu","year":"2011","journal-title":"Nature"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1186\/s13293-019-0225-y","article-title":"Sex-specific lipid molecular signatures in obesity-associated metabolic dysfunctions revealed by lipidomic characterization in ob\/ob mouse","volume":"10","author":"Helguero","year":"2019","journal-title":"Biol. Sex. Differ."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"321","DOI":"10.1016\/j.cmet.2006.03.007","article-title":"The ratio of phosphatidylcholine to phosphatidylethanolamine influences membrane integrity and steatohepatitis","volume":"3","author":"Li","year":"2006","journal-title":"Cell Metab."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"27043","DOI":"10.1074\/jbc.273.42.27043","article-title":"Biochemical and evolutionary significance of phospholipid methylation","volume":"273","author":"Walkey","year":"1998","journal-title":"J. Biol. Chem."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"5081","DOI":"10.1016\/S0021-9258(19)89217-7","article-title":"The role of cholesterol in the activity of reconstituted ca-atpase vesicles containing unsaturated phosphatidylethanolamine","volume":"261","author":"Cheng","year":"1986","journal-title":"J. Biol. Chem."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"37030","DOI":"10.1074\/jbc.M405195200","article-title":"Enrichment of endoplasmic reticulum with cholesterol inhibits sarcoplasmic-endoplasmic reticulum calcium atpase-2b activity in parallel with increased order of membrane lipids: Implications for depletion of endoplasmic reticulum calcium stores and apoptosis in cholesterol-loaded macrophages","volume":"279","author":"Li","year":"2004","journal-title":"J. Biol. Chem."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1126\/science.1175088","article-title":"An er-mitochondria tethering complex revealed by a synthetic biology screen","volume":"325","author":"Kornmann","year":"2009","journal-title":"Science"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"a004424","DOI":"10.1101\/cshperspect.a004424","article-title":"Er stress and its functional link to mitochondria: Role in cell survival and death","volume":"3","author":"Malhotra","year":"2011","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"7248","DOI":"10.1016\/S0021-9258(19)39106-9","article-title":"Phospholipid synthesis in a membrane fraction associated with mitochondria","volume":"265","author":"Vance","year":"1990","journal-title":"J. Biol. Chem."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"358","DOI":"10.2337\/db15-0659","article-title":"Skeletal muscle phospholipid metabolism regulates insulin sensitivity and contractile function","volume":"65","author":"Funai","year":"2016","journal-title":"Diabetes"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"1229","DOI":"10.1172\/JCI65726","article-title":"Muscle lipogenesis balances insulin sensitivity and strength through calcium signaling","volume":"123","author":"Funai","year":"2013","journal-title":"J. Clin. Investig."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"108","DOI":"10.1016\/j.diff.2016.05.007","article-title":"Skeletal muscle fibroblasts in health and disease","volume":"92","author":"Chapman","year":"2016","journal-title":"Differentiation"},{"key":"ref_45","doi-asserted-by":"crossref","unstructured":"Paradies, G., Paradies, V., Ruggiero, F.M., and Petrosillo, G. (2019). Role of cardiolipin in mitochondrial function and dynamics in health and disease: Molecular and pharmacological aspects. Cells, 8.","DOI":"10.3390\/cells8070728"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"67","DOI":"10.1016\/j.tcb.2017.08.011","article-title":"Phosphatidic acid and cardiolipin coordinate mitochondrial dynamics","volume":"28","author":"Kameoka","year":"2018","journal-title":"Trends Cell Biol."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"856","DOI":"10.1038\/ncb3560","article-title":"Molecular basis of selective mitochondrial fusion by heterotypic action between opa1 and cardiolipin","volume":"19","author":"Ban","year":"2017","journal-title":"Nat. Cell Biol."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1016\/j.biochi.2017.08.013","article-title":"Role of cardiolipin in stability of integral membrane proteins","volume":"142","author":"Musatov","year":"2017","journal-title":"Biochimie"},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1016\/j.tibs.2011.09.003","article-title":"The complexity of cardiolipin in health and disease","volume":"37","author":"Claypool","year":"2012","journal-title":"Trends Biochem. Sci."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"90","DOI":"10.3389\/fcell.2017.00090","article-title":"Role of cardiolipin in mitochondrial signaling pathways","volume":"5","author":"Dudek","year":"2017","journal-title":"Front. Cell Dev. Biol."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"1925","DOI":"10.1089\/ars.2013.5280","article-title":"Cardiolipin and mitochondrial function in health and disease","volume":"20","author":"Paradies","year":"2014","journal-title":"Antioxid. Redox Signal."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"e14037","DOI":"10.14814\/phy2.14037","article-title":"Blood cytokine patterns suggest a modest inflammation phenotype in subjects with long-chain fatty acid oxidation disorders","volume":"7","author":"McCoin","year":"2019","journal-title":"Physiol. Rep."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"1978","DOI":"10.2174\/092986709788682182","article-title":"Inhibition of sphingomyelin hydrolysis: Targeting the lipid mediator ceramide as a key regulator of cellular fate","volume":"16","author":"Claus","year":"2009","journal-title":"Curr. Med. Chem."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1111\/j.1476-5381.2009.00281.x","article-title":"Sphingolipids in inflammation: Pathological implications and potential therapeutic targets","volume":"158","author":"Nixon","year":"2009","journal-title":"Br. J. Pharmacol."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"1214","DOI":"10.1212\/WNL.0000000000002004","article-title":"Interictal, circulating sphingolipids in women with episodic migraine: A case-control study","volume":"85","author":"Peterlin","year":"2015","journal-title":"Neurology"},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"1239","DOI":"10.1016\/j.bbabio.2010.01.021","article-title":"Ceramide channels and their role in mitochondria-mediated apoptosis","volume":"1797","author":"Colombini","year":"2010","journal-title":"Biochim. Biophys. Acta"},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"789","DOI":"10.1042\/BJ20111626","article-title":"Ceramide synthases at the centre of sphingolipid metabolism and biology","volume":"441","author":"Mullen","year":"2012","journal-title":"Biochem. J."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"1388","DOI":"10.1096\/fasebj.10.12.8903509","article-title":"Sphingolipid metabolism and cell growth regulation","volume":"10","author":"Spiegel","year":"1996","journal-title":"FASEB J."},{"key":"ref_59","doi-asserted-by":"crossref","unstructured":"Bandet, C.L., Tan-Chen, S., Bourron, O., Le Stunff, H., and Hajduch, E. (2019). Sphingolipid metabolism: New insight into ceramide-induced lipotoxicity in muscle cells. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20030479"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1016\/j.plipres.2014.01.004","article-title":"Ceramide: A simple sphingolipid with unique biophysical properties","volume":"54","author":"Castro","year":"2014","journal-title":"Prog. Lipid Res."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1038\/nrm2329","article-title":"Principles of bioactive lipid signalling: Lessons from sphingolipids","volume":"9","author":"Hannun","year":"2008","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"ref_62","doi-asserted-by":"crossref","unstructured":"Bouscary, A., Quessada, C., Rene, F., Spedding, M., Turner, B.J., Henriques, A., Ngo, S.T., and Loeffler, J.P. (2020). Sphingolipids metabolism alteration in the central nervous system: Amyotrophic lateral sclerosis (als) and other neurodegenerative diseases. Semin. Cell Dev. Biol., 112.","DOI":"10.1016\/j.semcdb.2020.10.008"},{"key":"ref_63","doi-asserted-by":"crossref","unstructured":"Moskot, M., Bochenska, K., Jakobkiewicz-Banecka, J., Banecki, B., and Gabig-Ciminska, M. (2018). Abnormal sphingolipid world in inflammation specific for lysosomal storage diseases and skin disorders. Int. J. Mol. Sci., 19.","DOI":"10.3390\/ijms19010247"},{"key":"ref_64","doi-asserted-by":"crossref","unstructured":"Sigruener, A., Kleber, M.E., Heimerl, S., Liebisch, G., Schmitz, G., and Maerz, W. (2014). Glycerophospholipid and sphingolipid species and mortality: The ludwigshafen risk and cardiovascular health (luric) study. PLoS ONE, 9.","DOI":"10.1371\/journal.pone.0085724"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"928","DOI":"10.1093\/hmg\/ddy403","article-title":"Mitochondrial energetics is impaired in very long-chain acyl-coa dehydrogenase deficiency and can be rescued by treatment with mitochondria-targeted electron scavengers","volume":"28","author":"Seminotti","year":"2019","journal-title":"Hum. Mol. Genet."},{"key":"ref_66","doi-asserted-by":"crossref","unstructured":"Pujol-Lereis, L.M. (2019). Alteration of sphingolipids in biofluids: Implications for neurodegenerative diseases. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20143564"},{"key":"ref_67","doi-asserted-by":"crossref","unstructured":"Mielke, M.M., Maetzler, W., Haughey, N.J., Bandaru, V.V., Savica, R., Deuschle, C., Gasser, T., Hauser, A.K., Graber-Sultan, S., and Schleicher, E. (2013). Plasma ceramide and glucosylceramide metabolism is altered in sporadic parkinson\u2019s disease and associated with cognitive impairment: A pilot study. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0073094"},{"key":"ref_68","doi-asserted-by":"crossref","unstructured":"Pujol-Lereis, L.M., Liebisch, G., Schick, T., Lin, Y., Grassmann, F., Uchida, K., Zipfel, P.F., Fauser, S., Skerka, C., and Weber, B.H.F. (2018). Evaluation of serum sphingolipids and the influence of genetic risk factors in age-related macular degeneration. PLoS ONE, 13.","DOI":"10.1371\/journal.pone.0200739"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"2271","DOI":"10.1093\/brain\/awu139","article-title":"Cerebrospinal fluid ceramides from patients with multiple sclerosis impair neuronal bioenergetics","volume":"137","author":"Vidaurre","year":"2014","journal-title":"Brain"},{"key":"ref_70","doi-asserted-by":"crossref","unstructured":"Natarajan, S.K., and Ibdah, J.A. (2018). Role of 3-hydroxy fatty acid-induced hepatic lipotoxicity in acute fatty liver of pregnancy. Int. J. Mol. Sci., 19.","DOI":"10.3390\/ijms19010322"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"930","DOI":"10.1016\/j.neuint.2010.03.025","article-title":"Long-chain 3-hydroxy fatty acids accumulating in lchad and mtp deficiencies induce oxidative stress in rat brain","volume":"56","author":"Tonin","year":"2010","journal-title":"Neurochem. Int."}],"container-title":["Cells"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2073-4409\/10\/5\/1239\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T06:03:18Z","timestamp":1760162598000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2073-4409\/10\/5\/1239"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,5,18]]},"references-count":71,"journal-issue":{"issue":"5","published-online":{"date-parts":[[2021,5]]}},"alternative-id":["cells10051239"],"URL":"https:\/\/doi.org\/10.3390\/cells10051239","relation":{},"ISSN":["2073-4409"],"issn-type":[{"value":"2073-4409","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,5,18]]}}}