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The most common FAODs are characterized by the accumulation of medium-chain FAs and long-chain (3-hydroxy) FAs (and their carnitine derivatives), respectively. These deregulations are associated with lipotoxicity which affects several organs and potentially leads to life-threatening complications and comorbidities. Changes in the lipidome have been associated with several diseases, including some IEMs. In FAODs, the alteration of acylcarnitines (CARs) and FA profiles have been reported in patients and animal models, but changes in polar and neutral lipid profile are still scarcely studied. In this review, we present the main findings on FA and CAR profile changes associated with FAOD pathogenesis, their correlation with oxidative damage, and the consequent disturbance of mitochondrial homeostasis. Moreover, alterations in polar and neutral lipid classes and lipid species identified so far and their possible role in FAODs are discussed. We highlight the need of mass-spectrometry-based lipidomic studies to understand (epi)lipidome remodelling in FAODs, thus allowing to elucidate the pathophysiology and the identification of possible biomarkers for disease prognosis and an evaluation of therapeutic efficacy.<\/jats:p>","DOI":"10.3390\/ijms232213933","type":"journal-article","created":{"date-parts":[[2022,11,14]],"date-time":"2022-11-14T02:29:32Z","timestamp":1668392972000},"page":"13933","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":90,"title":["Mitochondrial Fatty Acid \u03b2-Oxidation Disorders: From Disease to Lipidomic Studies\u2014A Critical Review"],"prefix":"10.3390","volume":"23","author":[{"given":"In\u00eas M. 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Disord."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1067\/S0022-3476(03)00292-0","article-title":"MS\/MS-Based Newborn and Family Screening Detects Asymptomatic Patients with Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency","volume":"143","author":"Spiekerkoetter","year":"2003","journal-title":"J. Pediatr."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"521","DOI":"10.1007\/s10571-020-00955-7","article-title":"Evidence That Oxidative Disbalance and Mitochondrial Dysfunction Are Involved in the Pathophysiology of Fatty Acid Oxidation Disorders","volume":"42","author":"Ribas","year":"2020","journal-title":"Cell. Mol. Neurobiol."},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Ruiz-Sala, P., and Pe\u00f1a-Quintana, L. (2021). Biochemical Markers for the Diagnosis of Mitochondrial Fatty Acid Oxidation Diseases. J. Clin. 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Interact."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"2191","DOI":"10.1007\/s00216-019-02241-y","article-title":"Lipidomics from Sample Preparation to Data Analysis: A Primer","volume":"412","year":"2020","journal-title":"Anal. Bioanal. Chem."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1007\/s10545-017-0114-7","article-title":"Plasma Lipidomics as a Diagnostic Tool for Peroxisomal Disorders","volume":"41","author":"Herzog","year":"2018","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"106654","DOI":"10.1016\/j.microc.2021.106654","article-title":"Novel LC-MS Tools for Diagnosing Inborn Errors of Metabolism","volume":"170","year":"2021","journal-title":"Microchem. J."},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Ismail, I.T., Showalter, M.R., and Fiehn, O. (2019). Inborn Errors of Metabolism in the Era of Untargeted Metabolomics and Lipidomics. Metabolites, 9.","DOI":"10.3390\/metabo9100242"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"469","DOI":"10.1007\/s10545-010-9061-2","article-title":"A General Introduction to the Biochemistry of Mitochondrial Fatty Acid \u03b2-Oxidation","volume":"33","author":"Houten","year":"2010","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1146\/annurev-physiol-021115-105045","article-title":"The Biochemistry and Physiology of Mitochondrial Fatty Acid \u03b2-Oxidation and Its Genetic Disorders","volume":"78","author":"Houten","year":"2016","journal-title":"Annu. Rev. Physiol."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1016\/S0009-8981(02)00182-1","article-title":"Strategies for the Diagnosis of Mitochondrial Fatty Acid \u03b2-Oxidation Disorders","volume":"323","author":"Sim","year":"2002","journal-title":"Clin. Chim. Acta"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"140","DOI":"10.1016\/j.spen.2008.05.008","article-title":"Mitochondrial Fatty-Acid Oxidation Disorders","volume":"15","author":"Kompare","year":"2008","journal-title":"Semin. Pediatr. Neurol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"20023","DOI":"10.1074\/jbc.R115.663260","article-title":"Physiological Consequences of Compartmentalized Acyl-CoA Metabolism","volume":"290","author":"Cooper","year":"2015","journal-title":"J. Biol. Chem."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"e00313","DOI":"10.1042\/BSR20150295","article-title":"Combined defects in oxidative phosphorylation and fatty acid \u03b2-oxidation in mitochondrial disease","volume":"36","author":"McKenzie","year":"2016","journal-title":"Biosci. Rep."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"2422","DOI":"10.1016\/j.bbamcr.2016.01.023","article-title":"Carnitine Transport and Fatty Acid Oxidation","volume":"1863","author":"Longo","year":"2016","journal-title":"Biochim. Biophys. Acta (BBA)-Mol. Cell Res."},{"key":"ref_20","first-page":"1","article-title":"Fatty Acid Import into Mitochondria","volume":"1486","author":"Kerner","year":"2000","journal-title":"Biochim. Biophys. Acta (BBA)-Mol. Cell Biol. Lipids"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1007\/s10545-010-9104-8","article-title":"The Enzymology of Mitochondrial Fatty Acid Beta-Oxidation and Its Application to Follow-up Analysis of Positive Neonatal Screening Results","volume":"33","author":"Wanders","year":"2010","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1016\/j.mito.2018.02.009","article-title":"Mitochondrial \u03b2-Oxidation of Saturated Fatty Acids in Humans","volume":"46","year":"2019","journal-title":"Mitochondrion"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"462","DOI":"10.1046\/j.1432-1033.2003.03947.x","article-title":"Mitochondrial \u03b2-Oxidation","volume":"271","author":"Bartlett","year":"2004","journal-title":"Eur. J. Biochem."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"1375","DOI":"10.1517\/21678707.2015.1092869","article-title":"A Short Introduction to Acyl-CoA Dehydrogenases; Deficiencies and Novel Treatment Strategies","volume":"3","author":"Lund","year":"2015","journal-title":"Expert Opin. Orphan Drugs"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"442","DOI":"10.1023\/A:1005504223140","article-title":"Disorders of Mitochondrial Fatty Acyl-CoA Beta-Oxidation","volume":"22","author":"Wanders","year":"1999","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1146\/annurev.physiol.64.082201.154705","article-title":"Fatty Acid Oxidation Disorders","volume":"64","author":"Rinaldo","year":"2002","journal-title":"Annu. Rev. Physiol."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"e00281","DOI":"10.1042\/BSR20150240","article-title":"Mitochondrial Dysfunction in Fatty Acid Oxidation Disorders: Insights from Human and Animal Studies","volume":"36","author":"Wajner","year":"2016","journal-title":"Biosci. Rep."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1007\/s10545-013-9611-5","article-title":"Pathophysiology of Fatty Acid Oxidation Disorders and Resultant Phenotypic Variability","volume":"36","author":"Olpin","year":"2013","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1007\/s11154-020-09568-3","article-title":"Clinical Manifestations and Management of Fatty Acid Oxidation Disorders","volume":"21","author":"Merritt","year":"2020","journal-title":"Rev. Endocr. Metab. Disord."},{"key":"ref_30","doi-asserted-by":"crossref","unstructured":"Blau, N., Duran, M., Gibson, K.M., and Dionisi-Vici, C. (2014). Physician\u2019s Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases, Springer. [2nd ed.].","DOI":"10.1007\/978-3-642-40337-8"},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"473","DOI":"10.21037\/atm.2018.10.57","article-title":"Fatty Acid Oxidation Disorders","volume":"6","author":"Merritt","year":"2018","journal-title":"Ann. Transl. Med."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"64","DOI":"10.1016\/j.ymgme.2019.04.001","article-title":"Clinical and Biochemical Outcome of Patients with Very Long-Chain Acyl-CoA Dehydrogenase Deficiency","volume":"127","author":"Rovelli","year":"2019","journal-title":"Mol. Genet. Metab."},{"key":"ref_33","doi-asserted-by":"crossref","unstructured":"Loeber, J.G., Platis, D., Zetterstr\u00f6m, R.H., Almashanu, S., Boemer, F., Bonham, J.R., Borde, P., Brincat, I., Cheillan, D., and Dekkers, E. (2021). Neonatal Screening in Europe Revisited: An ISNS Perspective on the Current State and Developments since 2010. Int. J. Neonatal Screen., 7.","DOI":"10.3390\/ijns7010015"},{"key":"ref_34","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_35","doi-asserted-by":"crossref","first-page":"S133","DOI":"10.1007\/s10545-010-9048-z","article-title":"Four Years of Expanded Newborn Screening in Portugal with Tandem Mass Spectrometry","volume":"33","author":"Vilarinho","year":"2010","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1038\/s10038-018-0527-7","article-title":"Management and Diagnosis of Mitochondrial Fatty Acid Oxidation Disorders: Focus on Very-Long-Chain Acyl-CoA Dehydrogenase Deficiency","volume":"64","author":"Yamada","year":"2019","journal-title":"J. Hum. Genet."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"313","DOI":"10.12997\/jla.2020.9.3.313","article-title":"Mitochondrial Fatty Acid Oxidation Disorders: Laboratory Diagnosis, Pathogenesis, and the Complicated Route to Treatment","volume":"9","author":"Wanders","year":"2020","journal-title":"J. Lipid Atheroscler."},{"key":"ref_38","unstructured":"Saudubray, J.-M., Baumgartner, M.R., and Walter, J. (2016). Disorders of Mitochondrial Fatty Acid Oxidation & Riboflavin Metabolism. Inborn Metabolic Diseases: Diagnosis and Treatment, Springer."},{"key":"ref_39","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_40","doi-asserted-by":"crossref","first-page":"S50","DOI":"10.1016\/j.ejphar.2011.05.085","article-title":"Fatty Acids and Inflammation: The Cutting Edge between Food and Pharma","volume":"668","author":"Calder","year":"2011","journal-title":"Eur. J. Pharmacol."},{"key":"ref_41","doi-asserted-by":"crossref","unstructured":"Radzikowska, U., Rinaldi, A.O., \u00c7elebi S\u00f6zener, Z., Karaguzel, D., Wojcik, M., Cypryk, K., Akdis, M., Akdis, C.A., and Sokolowska, M. (2019). The Influence of Dietary Fatty Acids on Immune Responses. Nutrients, 11.","DOI":"10.3390\/nu11122990"},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1007\/s40139-017-0148-4","article-title":"Mitochondrial Fatty Acid Oxidation Disorders Associated with Cardiac Disease","volume":"5","author":"Goldstein","year":"2017","journal-title":"Curr. Pathobiol. Rep."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1007\/s10545-010-9121-7","article-title":"Mitochondrial Fatty Acid Oxidation Disorders: Pathophysiological Studies in Mouse Models","volume":"33","author":"Spiekerkoetter","year":"2010","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"62","DOI":"10.1016\/j.talanta.2015.02.041","article-title":"Oxidized Phosphatidylcholines Suggest Oxidative Stress in Patients with Medium-Chain Acyl-CoA Dehydrogenase Deficiency","volume":"139","author":"Najdekr","year":"2015","journal-title":"Talanta"},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"519","DOI":"10.1016\/j.neuint.2009.02.009","article-title":"Medium-Chain Fatty Acids Accumulating in MCAD Deficiency Elicit Lipid and Protein Oxidative Damage and Decrease Non-Enzymatic Antioxidant Defenses in Rat Brain","volume":"54","author":"Schuck","year":"2009","journal-title":"Neurochem. Int."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1016\/j.ijdevneu.2012.03.238","article-title":"Disruption of Redox Homeostasis in Cerebral Cortex of Developing Rats by Acylcarnitines Accumulating in Medium-Chain Acyl-CoA Dehydrogenase Deficiency","volume":"30","author":"Tonin","year":"2012","journal-title":"Int. J. Dev. Neurosci."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"1261","DOI":"10.1080\/10715760701687109","article-title":"Oxidative Stress Induction by Cis-4-Decenoic Acid: Relevance for MCAD Deficiency","volume":"41","author":"Schuck","year":"2007","journal-title":"Free Radic. Res."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1007\/s11010-011-1119-4","article-title":"Toxicity of Octanoate and Decanoate in Rat Peripheral Tissues: Evidence of Bioenergetic Dysfunction and Oxidative Damage Induction in Liver and Skeletal Muscle","volume":"361","author":"Scaini","year":"2012","journal-title":"Mol. Cell. Biochem."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"1363","DOI":"10.1016\/j.bbabio.2016.05.007","article-title":"Cis-4-Decenoic and Decanoic Acids Impair Mitochondrial Energy, Redox and Ca2+ Homeostasis and Induce Mitochondrial Permeability Transition Pore Opening in Rat Brain and Liver: Possible Implications for the Pathogenesis of MCAD Deficiency","volume":"1857","author":"Amaral","year":"2016","journal-title":"Biochim. Biophys. Acta (BBA)-Bioenerg."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/j.brainres.2004.10.010","article-title":"Inhibition of Energy Metabolism in Cerebral Cortex of Young Rats by the Medium-Chain Fatty Acids Accumulating in MCAD Deficiency","volume":"1030","author":"Maria","year":"2004","journal-title":"Brain Res."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"620","DOI":"10.1016\/j.bbabio.2015.04.003","article-title":"Uncoupling, Metabolic Inhibition and Induction of Mitochondrial Permeability Transition in Rat Liver Mitochondria Caused by the Major Long-Chain Hydroxyl Monocarboxylic Fatty Acids Accumulating in LCHAD Deficiency","volume":"1847","author":"Hickmann","year":"2015","journal-title":"Biochim. Biophys. Acta (BBA)-Bioenerg."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.tiv.2016.06.007","article-title":"Disturbance of Mitochondrial Functions Provoked by the Major Long-Chain 3-Hydroxylated Fatty Acids Accumulating in MTP and LCHAD Deficiencies in Skeletal Muscle","volume":"36","author":"Cecatto","year":"2016","journal-title":"Toxicol. In Vitro"},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"825","DOI":"10.1016\/j.lfs.2010.04.003","article-title":"Disturbance of Mitochondrial Energy Homeostasis Caused by the Metabolites Accumulating in LCHAD and MTP Deficiencies in Rat Brain","volume":"86","author":"Tonin","year":"2010","journal-title":"Life Sci."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"4714","DOI":"10.1111\/febs.13526","article-title":"Deregulation of Mitochondrial Functions Provoked by Long-Chain Fatty Acid Accumulating in Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase and Mitochondrial Permeability Transition Deficiencies in Rat Heart\u2014Mitochondrial Permeability Transition Pore Opening as a Potential Contributing Pathomechanism of Cardiac Alterations in These Disorders","volume":"282","author":"Cecatto","year":"2015","journal-title":"FEBS J."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1007\/s10863-012-9481-9","article-title":"Long-Chain 3-Hydroxy Fatty Acids Accumulating in Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase and Mitochondrial Trifunctional Protein Deficiencies Uncouple Oxidative Phosphorylation in Heart Mitochondria","volume":"45","author":"Tonin","year":"2013","journal-title":"J. Bioenerg. Biomembr."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"7678","DOI":"10.1002\/jcb.27115","article-title":"High Vulnerability of the Heart and Liver to 3-Hydroxypalmitic Acid\u2013Induced Disruption of Mitochondrial Functions in Intact Cell Systems","volume":"119","author":"Cecatto","year":"2018","journal-title":"J. Cell. Biochem."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"1658","DOI":"10.1016\/j.bbadis.2014.06.011","article-title":"Mitochondrial Bioenergetics Deregulation Caused by Long-Chain 3-Hydroxy Fatty Acids Accumulating in LCHAD and MTP Deficiencies in Rat Brain: A Possible Role of MPTP Opening as a Pathomechanism in These Disorders?","volume":"1842","author":"Tonin","year":"2014","journal-title":"Biochim. Biophys. Acta (BBA)-Mol. Basis Dis."},{"key":"ref_58","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."},{"key":"ref_59","doi-asserted-by":"crossref","first-page":"108206","DOI":"10.1016\/j.abb.2019.108206","article-title":"Oxidative Damage in Mitochondrial Fatty Acids Oxidation Disorders Patients and the in Vitro Effect of L-Carnitine on DNA Damage Induced by the Accumulated Metabolites","volume":"679","author":"Guerreiro","year":"2020","journal-title":"Arch. Biochem. Biophys."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.mito.2019.09.008","article-title":"Disturbance of Mitochondrial Functions Associated with Permeability Transition Pore Opening Induced by Cis-5-Tetradecenoic and Myristic Acids in Liver of Adolescent Rats","volume":"50","author":"Cecatto","year":"2020","journal-title":"Mitochondrion"},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"1437","DOI":"10.1111\/febs.14419","article-title":"Metabolite Accumulation in VLCAD Deficiency Markedly Disrupts Mitochondrial Bioenergetics and Ca2+ Homeostasis in the Heart","volume":"285","author":"Cecatto","year":"2018","journal-title":"FEBS J."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"104665","DOI":"10.1016\/j.tiv.2019.104665","article-title":"Impairment of Mitochondrial Bioenergetics and Permeability Transition Induction Caused by Major Long-Chain Fatty Acids Accumulating in VLCAD Deficiency in Skeletal Muscle as Potential Pathomechanisms of Myopathy","volume":"62","author":"Cecatto","year":"2020","journal-title":"Toxicol. In Vitro"},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"25","DOI":"10.1007\/s11745-013-3865-4","article-title":"Monounsaturated 14:1n-9 and 16:1n-9 Fatty Acids but Not 18:1n-9 Induce Apoptosis and Necrosis in Murine HL-1 Cardiomyocytes","volume":"49","author":"Hoffmann","year":"2014","journal-title":"Lipids"},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1038\/nrendo.2015.129","article-title":"Acylcarnitines\u2014Old Actors Auditioning for New Roles in Metabolic Physiology","volume":"11","author":"McCoin","year":"2015","journal-title":"Nat. Rev. Endocrinol."},{"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","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_67","doi-asserted-by":"crossref","first-page":"839","DOI":"10.1194\/jlr.E400004-JLR200","article-title":"A Comprehensive Classification System for Lipids1","volume":"46","author":"Fahy","year":"2005","journal-title":"J. Lipid Res."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"S9","DOI":"10.1194\/jlr.R800095-JLR200","article-title":"Update of the LIPID MAPS Comprehensive Classification System for Lipids1","volume":"50","author":"Fahy","year":"2009","journal-title":"J. Lipid Res."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"1539","DOI":"10.1194\/jlr.S120001025","article-title":"Update on LIPID MAPS Classification, Nomenclature, and Shorthand Notation for MS-Derived Lipid Structures","volume":"61","author":"Liebisch","year":"2020","journal-title":"J. Lipid Res."},{"key":"ref_70","first-page":"158857","article-title":"Systems Biology Approaches to Study Lipidomes in Health and Disease","volume":"1866","author":"Alves","year":"2021","journal-title":"Biochim. Biophys. Acta (BBA)-Mol. Cell Biol. Lipids"},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"115664","DOI":"10.1016\/j.trac.2019.115664","article-title":"Lipidomes in Health and Disease: Analytical Strategies and Considerations","volume":"120","author":"Wei","year":"2019","journal-title":"Trends Anal. Chem."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/S0009-8981(97)00130-7","article-title":"Plasma Free Fatty Acids in Mitochondrial Fatty Acid Oxidation Defects","volume":"267","author":"Divry","year":"1997","journal-title":"Clin. Chim. Acta"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1093\/clinchem\/44.3.463","article-title":"Simultaneous Analysis of Plasma Free Fatty Acids and Their 3-Hydroxy Analogs in Fatty Acid \u03b2-Oxidation Disorders","volume":"44","author":"Costa","year":"1998","journal-title":"Clin. Chem."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"117","DOI":"10.1016\/S0009-8981(01)00741-0","article-title":"A Sensitive and Simplified Method to Analyze Free Fatty Acids in Children with Mitochondrial Beta Oxidation Disorders Using Gas Chromatography\/Mass Spectrometry and Dried Blood Spots","volume":"316","author":"Kimura","year":"2002","journal-title":"Clin. Chim. Acta"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1023\/A:1010592232317","article-title":"Intermediates of Unsaturated Fatty Acid Oxidation Are Incorporated in Triglycerides but Not in Phospholipids in Tissues from Patients with Mitochondrial \u03b2-Oxidation Defects","volume":"24","author":"Onkenhout","year":"2001","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"266","DOI":"10.1016\/j.freeradbiomed.2019.03.036","article-title":"An Update on Lipid Oxidation and Inflammation in Cardiovascular Diseases","volume":"144","author":"Zhong","year":"2019","journal-title":"Free Radic. Biol. Med."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"1818","DOI":"10.1002\/eji.201545676","article-title":"The Regulation of Inflammation by Oxidized Phospholipids","volume":"46","author":"Freigang","year":"2016","journal-title":"Eur. J. Immunol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1023\/A:1025175606891","article-title":"Plasma and Erythrocyte Fatty Acid Concentrations in Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency","volume":"26","author":"Lund","year":"2003","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1023\/A:1005673828469","article-title":"Acylcarnitines in Plasma and Blood Spots of Patients with Long-Chain 3-Hydroxyacyl-Coenzyme A Dehydrogenase Defiency","volume":"23","author":"Kahler","year":"2000","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_80","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_81","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1023\/A:1005694712583","article-title":"Acylcarnitines in Fibroblasts of Patients with Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency and Other Fatty Acid Oxidation Disorders","volume":"23","author":"Shen","year":"2000","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_82","doi-asserted-by":"crossref","unstructured":"Alatibi, K.I., Hagenbuchner, J., Wehbe, Z., Karall, D., Ausserlechner, M.J., Vockley, J., Spiekerkoetter, U., Gr\u00fcnert, S.C., and Tucci, S. (2021). Different Lipid Signature in Fibroblasts of Long-Chain Fatty Acid Oxidation Disorders. Cells, 10.","DOI":"10.3390\/cells10051239"},{"key":"ref_83","doi-asserted-by":"crossref","unstructured":"Alatibi, K.I., Tholen, S., Wehbe, Z., Hagenbuchner, J., Karall, D., Ausserlechner, M.J., Schilling, O., Gr\u00fcnert, S.C., Vockley, J., and Tucci, S. (2021). Lipidomic and Proteomic Alterations Induced by Even and Odd Medium-Chain Fatty Acids on Fibroblasts of Long-Chain Fatty Acid Oxidation Disorders. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms221910556"},{"key":"ref_84","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 (BBA)-Biomembr."},{"key":"ref_85","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_86","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_87","doi-asserted-by":"crossref","first-page":"1442","DOI":"10.1016\/j.bbadis.2012.05.008","article-title":"Functions of Plasmalogen Lipids in Health and Disease","volume":"1822","author":"Braverman","year":"2012","journal-title":"Biochim. Biophys. Acta (BBA)-Mol. Basis Dis."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"2021","DOI":"10.2174\/092986709788682164","article-title":"Plasmalogens in Biological Systems: Their Role in Oxidative Processes in Biological Membranes, Their Contribution to Pathological Processes and Aging and Plasmalogen Analysis","volume":"16","author":"Lessig","year":"2009","journal-title":"Curr. Med. Chem."},{"key":"ref_89","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_90","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_91","doi-asserted-by":"crossref","unstructured":"Casares, D., Escrib\u00e1, P.V., and Rossell\u00f3, C.A. (2019). Membrane Lipid Composition: Effect on Membrane and Organelle Structure, Function and Compartmentalization and Therapeutic Avenues. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20092167"},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1038\/nrm2330","article-title":"Membrane Lipids: Where They Are and How They Behave","volume":"9","author":"Voelker","year":"2008","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"ref_93","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_94","doi-asserted-by":"crossref","first-page":"eaao0076","DOI":"10.1126\/science.aao0076","article-title":"Rhomboid Distorts Lipids to Break the Viscosity-Imposed Speed Limit of Membrane Diffusion","volume":"363","author":"Kreutzberger","year":"2019","journal-title":"Science"},{"key":"ref_95","doi-asserted-by":"crossref","unstructured":"Sezgin, E., Gutmann, T., Buhl, T., Dirkx, R., Grzybek, M., Coskun, \u00dc., Solimena, M., Simons, K., Levental, I., and Schwille, P. (2015). Adaptive Lipid Packing and Bioactivity in Membrane Domains. PLoS ONE, 10.","DOI":"10.1371\/journal.pone.0123930"},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"101068","DOI":"10.1016\/j.plipres.2020.101068","article-title":"Emerging Roles of Lysophospholipids in Health and Disease","volume":"80","author":"Tan","year":"2020","journal-title":"Prog. Lipid Res."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"4242","DOI":"10.1111\/febs.13418","article-title":"De Novo Fatty Acid Biosynthesis and Elongation in Very Long-Chain Acyl-CoA Dehydrogenase-Deficient Mice Supplemented with Odd or Even Medium-Chain Fatty Acids","volume":"282","author":"Tucci","year":"2015","journal-title":"FEBS J."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"1442","DOI":"10.1016\/j.bbadis.2015.04.009","article-title":"Sexual Dimorphism of Lipid Metabolism in Very Long-Chain Acyl-CoA Dehydrogenase Deficient (VLCAD\u2212\/\u2212) Mice in Response to Medium-Chain Triglycerides (MCT)","volume":"1852","author":"Tucci","year":"2015","journal-title":"Biochim. Biophys. Acta (BBA)-Mol. Basis Dis."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"598760","DOI":"10.3389\/fgene.2020.598760","article-title":"Physiological Perspectives on the Use of Triheptanoin as Anaplerotic Therapy for Long Chain Fatty Acid Oxidation Disorders","volume":"11","author":"Sklirou","year":"2021","journal-title":"Front. Genet."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"317","DOI":"10.1007\/s10545-017-0016-8","article-title":"Very Long-Chain Acyl-CoA Dehydrogenase (VLCAD-) Deficiency\u2013Studies on Treatment Effects and Long-Term Outcomes in Mouse Models","volume":"40","author":"Tucci","year":"2017","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_101","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_102","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1016\/0163-7827(94)00010-J","article-title":"The Regulation of Stearoyl-CoA Desaturase (SCD)","volume":"34","author":"Ntambi","year":"1995","journal-title":"Prog. Lipid Res."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"2150","DOI":"10.1093\/clinchem\/47.12.2150","article-title":"Age and Sex Dependency of Carnitine Concentration in Human Serum and Skeletal Muscle","volume":"47","author":"Opalka","year":"2001","journal-title":"Clin. Chem."},{"key":"ref_104","first-page":"1591","article-title":"The Fate of Medium-Chain Fatty Acids in Very Long-Chain Acyl-CoA Dehydrogenase Deficiency (VLCADD): A Matter of Sex?","volume":"1864","author":"Wehbe","year":"2019","journal-title":"Biochim. Biophys. Acta (BBA)-Mol. Cell Biol. Lipids"},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"1400","DOI":"10.1016\/j.bbrc.2004.09.198","article-title":"Involvement of ERK, a MAP Kinase, in the Production of TGF-\u03b2 by Macrophages Treated with Liposomes Composed of Phosphatidylserine","volume":"324","author":"Otsuka","year":"2004","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_106","doi-asserted-by":"crossref","first-page":"1071","DOI":"10.1074\/jbc.M003649200","article-title":"Loss of Phospholipid Asymmetry and Surface Exposure of Phosphatidylserine Is Required for Phagocytosis of Apoptotic Cells by Macrophages and Fibroblasts","volume":"276","author":"Fadok","year":"2001","journal-title":"J. Biol. Chem."},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"651","DOI":"10.1038\/nature05185","article-title":"Phosphoinositides in Cell Regulation and Membrane Dynamics","volume":"443","year":"2006","journal-title":"Nature"}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/23\/22\/13933\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:16:42Z","timestamp":1760145402000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/23\/22\/13933"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11,11]]},"references-count":107,"journal-issue":{"issue":"22","published-online":{"date-parts":[[2022,11]]}},"alternative-id":["ijms232213933"],"URL":"https:\/\/doi.org\/10.3390\/ijms232213933","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,11,11]]}}}