{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,24]],"date-time":"2026-04-24T15:39:23Z","timestamp":1777045163467,"version":"3.51.4"},"reference-count":84,"publisher":"Elsevier BV","issue":"6","license":[{"start":{"date-parts":[[2021,6,1]],"date-time":"2021-06-01T00:00:00Z","timestamp":1622505600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2021,6,1]],"date-time":"2021-06-01T00:00:00Z","timestamp":1622505600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2021,2,15]],"date-time":"2021-02-15T00:00:00Z","timestamp":1613347200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100012526","name":"Aarhus University Faculty of Health Sciences","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100012526","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100013220","name":"Ultragenyx Pharmaceutical Inc","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100013220","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease"],"published-print":{"date-parts":[[2021,6]]},"DOI":"10.1016\/j.bbadis.2021.166100","type":"journal-article","created":{"date-parts":[[2021,2,5]],"date-time":"2021-02-05T19:44:31Z","timestamp":1612554271000},"page":"166100","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":15,"title":["Bezafibrate activation of PPAR drives disturbances in mitochondrial redox bioenergetics and decreases the viability of cells from patients with VLCAD deficiency"],"prefix":"10.1016","volume":"1867","author":[{"given":"Martin","family":"Lund","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kathrine G.","family":"Andersen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Robert","family":"Heaton","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Iain P.","family":"Hargreaves","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Niels","family":"Gregersen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rikke K.J.","family":"Olsen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.bbadis.2021.166100_bb0005","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/0925-4439(93)90058-9","article-title":"Very long-chain acyl-CoA dehydrogenase-deficiency - identification of a new inborn error of mitochondrial fatty-acid oxidation in fibroblasts","volume":"1180","author":"Bertrand","year":"1993","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2021.166100_bb0010","doi-asserted-by":"crossref","first-page":"501","DOI":"10.1007\/s10545-009-9001-1","article-title":"Fatty acid oxidation disorders: outcome and long-term prognosis","volume":"33","author":"Wilcken","year":"2010","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.bbadis.2021.166100_bb0015","doi-asserted-by":"crossref","first-page":"9435","DOI":"10.1074\/jbc.M709135200","article-title":"Structural basis for substrate fatty acyl chain specificity: crystal structure of human very long-chain acyl-CoA dehydrogenase","volume":"283","author":"McAndrew","year":"2008","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2021.166100_bb0020","doi-asserted-by":"crossref","first-page":"441","DOI":"10.1093\/cvr\/cvt212","article-title":"Myocardial energy shortage and unmet anaplerotic needs in the fasted long-chain acyl-CoA dehydrogenase knockout mouse","volume":"100","author":"Bakermans","year":"2013","journal-title":"Cardiovasc. Res."},{"key":"10.1016\/j.bbadis.2021.166100_bb0025","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1093\/cvr\/cvx017","article-title":"Metabolism in cardiomyopathy: every substrate matters","volume":"113","author":"Ritterhoff","year":"2017","journal-title":"Cardiovasc. Res."},{"key":"10.1016\/j.bbadis.2021.166100_bb0030","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":"10.1016\/j.bbadis.2021.166100_bb0035","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 beta-oxidation","volume":"33","author":"Houten","year":"2010","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.bbadis.2021.166100_bb0040","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":"10.1016\/j.bbadis.2021.166100_bb0045","doi-asserted-by":"crossref","first-page":"479","DOI":"10.1086\/302261","article-title":"Clear correlation of genotype with disease phenotype in very\u2013long-chain acyl-CoA dehydrogenase deficiency","volume":"64","author":"Andresen","year":"1999","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.bbadis.2021.166100_bb0050","doi-asserted-by":"crossref","first-page":"5249","DOI":"10.1093\/hmg\/ddt382","article-title":"Impaired amino acid metabolism contributes to fasting-induced hypoglycemia in fatty acid oxidation defects","volume":"22","author":"Houten","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2021.166100_bb0055","doi-asserted-by":"crossref","first-page":"1669","DOI":"10.1016\/j.jchromb.2010.01.046","article-title":"Heat stress deteriorates mitochondrial \u03b2-oxidation of long-chain fatty acids in cultured fibroblasts with fatty acid \u03b2-oxidation disorders","volume":"878","author":"Li","year":"2010","journal-title":"J. Chromatogr. B"},{"key":"10.1016\/j.bbadis.2021.166100_bb0060","doi-asserted-by":"crossref","first-page":"97","DOI":"10.1097\/CND.0b013e3181903126","article-title":"Metabolic myopathies: update 2009","volume":"10","author":"van Adel","year":"2009","journal-title":"J. Clin. Neuromuscul. Dis."},{"key":"10.1016\/j.bbadis.2021.166100_bb0065","doi-asserted-by":"crossref","first-page":"e45429","DOI":"10.1371\/journal.pone.0045429","article-title":"Tissue-specific strategies of the very-long chain acyl-CoA dehydrogenase-deficient (VLCAD\u2212\/\u2212) mouse to compensate a defective fatty acid \u03b2-oxidation","volume":"7","author":"Tucci","year":"2012","journal-title":"PLoS One"},{"key":"10.1016\/j.bbadis.2021.166100_bb0070","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0147818","article-title":"Altered energetics of exercise explain risk of rhabdomyolysis in very long-chain acyl-CoA dehydrogenase deficiency","volume":"11","author":"Diekman","year":"2016","journal-title":"PLoS One"},{"key":"10.1016\/j.bbadis.2021.166100_bb0075","doi-asserted-by":"crossref","first-page":"324","DOI":"10.1016\/j.nmd.2009.02.007","article-title":"Diagnostic assessment and long-term follow-up of 13 patients with Very Long-Chain Acyl-Coenzyme A dehydrogenase (VLCAD) deficiency","volume":"19","author":"Laforet","year":"2009","journal-title":"Neuromuscul. Disord."},{"key":"10.1016\/j.bbadis.2021.166100_bb0080","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":"2018","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2021.166100_bb0085","first-page":"1","article-title":"A short introduction to acyl-CoA dehydrogenases; deficiencies and novel treatment strategies","author":"Lund","year":"2015","journal-title":"Expert Opin. Orphan Drugs"},{"key":"10.1016\/j.bbadis.2021.166100_bb0090","doi-asserted-by":"crossref","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":"10.1016\/j.bbadis.2021.166100_bb0095","series-title":"Application of an Image Cytometry Protocol for Cellular and Mitochondrial Phenotyping on Fibroblasts From Patients With Inherited Disorders","first-page":"1","author":"Fernandez-Guerra","year":"2015"},{"key":"10.1016\/j.bbadis.2021.166100_bb0100","doi-asserted-by":"crossref","first-page":"4699","DOI":"10.1111\/j.1742-4658.2010.07876.x","article-title":"Fasting-induced oxidative stress in very long chain acyl-CoA dehydrogenase-deficient mice","volume":"277","author":"Tucci","year":"2010","journal-title":"FEBS J."},{"issue":"Suppl","key":"10.1016\/j.bbadis.2021.166100_bb0105","doi-asserted-by":"crossref","first-page":"S31","DOI":"10.1016\/j.ymgme.2013.10.007","article-title":"Genetic and cellular modifiers of oxidative stress: what can we learn from fatty acid oxidation defects?","volume":"110","author":"Olsen","year":"2013","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.bbadis.2021.166100_bb0110","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1186\/s12915-016-0327-5","article-title":"Living on the edge: substrate competition explains loss of robustness in mitochondrial fatty-acid oxidation disorders","volume":"14","author":"van Eunen","year":"2016","journal-title":"BMC Biol."},{"key":"10.1016\/j.bbadis.2021.166100_bb0115","doi-asserted-by":"crossref","first-page":"2695","DOI":"10.1093\/hmg\/ddi303","article-title":"Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders","volume":"14","author":"Djouadi","year":"2005","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2021.166100_bb0120","doi-asserted-by":"crossref","first-page":"446","DOI":"10.1203\/01.PDR.0000083001.91588.BB","article-title":"Correction of fatty acid oxidation in carnitine palmitoyl transferase 2-deficient cultured skin fibroblasts by bezafibrate","volume":"54","author":"Djouadi","year":"2003","journal-title":"Pediatr. Res."},{"key":"10.1016\/j.bbadis.2021.166100_bb0125","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1007\/s10545-015-9871-3","article-title":"Mitochondrial trifunctional protein deficiency in human cultured fibroblasts: effects of bezafibrate","volume":"39","author":"Djouadi","year":"2016","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.bbadis.2021.166100_bb0130","doi-asserted-by":"crossref","first-page":"362","DOI":"10.1016\/j.braindev.2009.06.001","article-title":"Effect of heat stress and bezafibrate on mitochondrial \u03b2-oxidation: comparison between cultured cells from normal and mitochondrial fatty acid oxidation disorder children using in vitro probe acylcarnitine profiling assay","volume":"32","author":"Li","year":"2010","journal-title":"Brain and Development"},{"key":"10.1016\/j.bbadis.2021.166100_bb0135","doi-asserted-by":"crossref","first-page":"607","DOI":"10.1212\/WNL.0000000000000118","article-title":"Bezafibrate in skeletal muscle fatty acid oxidation disorders: a randomized clinical trial","volume":"82","author":"Orngreen","year":"2014","journal-title":"Neurology"},{"key":"10.1016\/j.bbadis.2021.166100_bb0140","doi-asserted-by":"crossref","first-page":"838","DOI":"10.1056\/NEJMc0806334","article-title":"Bezafibrate for an inborn mitochondrial Beta-oxidation defect","volume":"360","author":"Bonnefont","year":"2009","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.bbadis.2021.166100_bb0145","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1038\/clpt.2010.55","article-title":"Long-term follow-up of bezafibrate treatment in patients with the myopathic form of carnitine palmitoyltransferase 2 deficiency","volume":"88","author":"B.J. P, B. J, L. P, A. F, M. A, R. S, R. D, G.-L. S, V. A, B. A, E. B, B.J. L, D. F","year":"2010","journal-title":"Clin. Pharmacol. Ther."},{"key":"10.1016\/j.bbadis.2021.166100_bb0150","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1007\/s10545-014-9779-3","article-title":"No effect of bezafibrate in patients with CPTII and VLCAD deficiencies","volume":"38","author":"\u00d8rngreen","year":"2015","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.bbadis.2021.166100_bb0155","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1007\/s10545-014-9775-7","article-title":"Should the beneficial impact of bezafibrate on fatty acid oxidation disorders be questioned?","volume":"38","author":"Bastin","year":"2015","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.bbadis.2021.166100_bb0160","first-page":"55","article-title":"Open-label clinical trial of bezafibrate treatment in patients with fatty acid oxidation disorders in Japan","volume":"15","author":"Yamada","year":"2018","journal-title":"Mol. Genet. Metab. Rep."},{"key":"10.1016\/j.bbadis.2021.166100_bb0165","doi-asserted-by":"crossref","first-page":"409","DOI":"10.1146\/annurev.med.53.082901.104018","article-title":"The mechanisms of action of PPARs","volume":"53","author":"Berger","year":"2002","journal-title":"Annu. Rev. Med."},{"key":"10.1016\/j.bbadis.2021.166100_bb0170","doi-asserted-by":"crossref","first-page":"1818","DOI":"10.1111\/bph.12413","article-title":"Turn up the power \u2013 pharmacological activation of mitochondrial biogenesis in mouse models","volume":"171","author":"Komen","year":"2014","journal-title":"Br. J. Pharmacol."},{"key":"10.1016\/j.bbadis.2021.166100_bb0175","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1186\/1423-0127-19-32","article-title":"Effects of L-carnitine against oxidative stress in human hepatocytes: involvement of peroxisome proliferator-activated receptor alpha","volume":"19","author":"Li","year":"2012","journal-title":"J. Biomed. Sci."},{"key":"10.1016\/j.bbadis.2021.166100_bb0180","doi-asserted-by":"crossref","first-page":"15924","DOI":"10.1073\/pnas.0306981100","article-title":"Activation of peroxisome proliferator-activated receptor \u03b4 induces fatty acid \u03b2-oxidation in skeletal muscle and attenuates metabolic syndrome","volume":"100","author":"Tanaka","year":"2003","journal-title":"Proc. Natl. Acad. Sci."},{"key":"10.1016\/j.bbadis.2021.166100_bb0185","doi-asserted-by":"crossref","first-page":"526","DOI":"10.1093\/hmg\/ddr482","article-title":"Effect of bezafibrate treatment on late-onset mitochondrial myopathy in mice","volume":"21","author":"Yatsuga","year":"2012","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2021.166100_bb0190","doi-asserted-by":"crossref","first-page":"1725","DOI":"10.1042\/BST20160129","article-title":"Mitochondrial fission and fusion","volume":"44","author":"Lee","year":"2016","journal-title":"Biochem. Soc. Trans."},{"key":"10.1016\/j.bbadis.2021.166100_bb0195","doi-asserted-by":"crossref","first-page":"1","DOI":"10.3390\/diseases5010001","article-title":"Mitochondrial dynamics in mitochondrial diseases","volume":"5","author":"Su\u00e1rez-Rivero","year":"2017","journal-title":"Diseases"},{"key":"10.1016\/j.bbadis.2021.166100_bb0200","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1155\/2012\/371691","article-title":"Fatty acid accumulation and resulting PPAR activation in fibroblasts due to trifunctional protein deficiency","volume":"2012","author":"Wakabayashi","year":"2012","journal-title":"PPAR Res."},{"key":"10.1016\/j.bbadis.2021.166100_bb0205","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1620\/tjem.240.113","article-title":"Activation of PPAR\u03b1 by fatty acid accumulation enhances fatty acid degradation and sulfatide synthesis","volume":"240","author":"Yang","year":"2016","journal-title":"Tohoku J. Exp. Med."},{"key":"10.1016\/j.bbadis.2021.166100_bb0210","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":"10.1016\/j.bbadis.2021.166100_bb0215","doi-asserted-by":"crossref","first-page":"1273","DOI":"10.1097\/01.CCM.0000045201.10682.F6","article-title":"A young woman with persistent hypoglycemia, rhabdomyolysis, and coma: recognizing fatty acid oxidation defects in adults","volume":"31","author":"Kluge","year":"2003","journal-title":"Crit. Care Med."},{"key":"10.1016\/j.bbadis.2021.166100_bb0220","doi-asserted-by":"crossref","first-page":"989","DOI":"10.1038\/gim.2015.22","article-title":"Fatty acid oxidation flux predicts the clinical severity of VLCAD deficiency","volume":"17","author":"Diekman","year":"2015","journal-title":"Genet. Med."},{"key":"10.1016\/j.bbadis.2021.166100_bb0225","doi-asserted-by":"crossref","DOI":"10.3390\/ijns3010002","article-title":"Fibroblast fatty-acid oxidation flux assays stratify risk in newborns with presumptive-positive results on screening for very-long chain acyl-CoA dehydrogenase deficiency","volume":"3","author":"Olpin","year":"2017","journal-title":"Int. J. Neonatal Screen."},{"key":"10.1016\/j.bbadis.2021.166100_bb0230","doi-asserted-by":"crossref","first-page":"198","DOI":"10.1006\/abio.1999.4022","article-title":"mRNA quantification by real time TaqMan polymerase chain reaction: validation and comparison with RNase protection","volume":"269","author":"Wang","year":"1999","journal-title":"Anal. Biochem."},{"key":"10.1016\/j.bbadis.2021.166100_bb0235","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1677\/jme.0.0250169","article-title":"Absolute quantification of mRNA using real-time reverse transcription polymerase chain reaction assays","volume":"25","author":"Bustin","year":"2000","journal-title":"J. Mol. Endocrinol."},{"key":"10.1016\/j.bbadis.2021.166100_bb0240","doi-asserted-by":"crossref","first-page":"4350","DOI":"10.1073\/pnas.76.9.4350","article-title":"Electrophoretic transfer of proteins from polyacrylamide gels to nitrocellulose sheets: procedure and some applications","volume":"76","author":"Towbin","year":"1979","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"10.1016\/j.bbadis.2021.166100_bb0245","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1016\/j.jneumeth.2008.05.003","article-title":"The use of total protein stains as loading controls: an alternative to high-abundance single-protein controls in semi-quantitative immunoblotting","volume":"172","author":"Aldridge","year":"2008","journal-title":"J. Neurosci. Methods"},{"key":"10.1016\/j.bbadis.2021.166100_bb0250","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1080\/15376510802305047","article-title":"Decreased ubiquinone availability and impaired mitochondrial cytochrome oxidase activity associated with statin treatment","volume":"19","author":"Duncan","year":"2009","journal-title":"Toxicol. Mech. Methods"},{"key":"10.1016\/j.bbadis.2021.166100_bb0255","doi-asserted-by":"crossref","first-page":"597","DOI":"10.1042\/bj1140597","article-title":"The kinetic properties of citrate synthase from rat liver mitochondria","volume":"114","author":"Shepherd","year":"1969","journal-title":"Biochem. J."},{"key":"10.1016\/j.bbadis.2021.166100_bb0260","doi-asserted-by":"crossref","first-page":"729","DOI":"10.1373\/clinchem.2006.078873","article-title":"Spectrophotometric assay for complex I of the respiratory chain in tissue samples and cultured fibroblasts","volume":"53","author":"Janssen","year":"2007","journal-title":"Clin. Chem."},{"key":"10.1016\/j.bbadis.2021.166100_bb0265","doi-asserted-by":"crossref","first-page":"681","DOI":"10.1016\/j.mad.2007.10.002","article-title":"Rapid neonatal weight gain in rats results in a renal ubiquinone (CoQ) deficiency associated with premature death","volume":"128","author":"Shelley","year":"2007","journal-title":"Mech. Ageing Dev."},{"key":"10.1016\/j.bbadis.2021.166100_bb0270","doi-asserted-by":"crossref","first-page":"1166","DOI":"10.1097\/00004647-199711000-00005","article-title":"Activity of mitochondrial respiratory chain enzymes after transient focal ischemia in the rat","volume":"17","author":"Canevari","year":"1997","journal-title":"J. Cereb. Blood Flow Metab."},{"key":"10.1016\/j.bbadis.2021.166100_bb0275","doi-asserted-by":"crossref","first-page":"430","DOI":"10.1002\/cyto.a.22032","article-title":"A novel and rapid apoptosis assay based on thiol redox status","volume":"81","author":"Skindersoe","year":"2012","journal-title":"Cytom. Part A"},{"key":"10.1016\/j.bbadis.2021.166100_bb0280","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1002\/cyto.a.22410","article-title":"Comparison of three thiol probes for determination of apoptosis-related changes in cellular redox status","volume":"85","author":"Skindersoe","year":"2014","journal-title":"Cytom. Part A"},{"key":"10.1016\/j.bbadis.2021.166100_bb0285","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1007\/s12013-013-9750-1","article-title":"Citric acid cycle and role of its intermediates in metabolism","volume":"68","author":"Akram","year":"2014","journal-title":"Cell Biochem. Biophys."},{"key":"10.1016\/j.bbadis.2021.166100_bb0290","doi-asserted-by":"crossref","first-page":"3317","DOI":"10.1016\/j.bbagen.2012.10.018","article-title":"Mitochondrial glutathione features, regulation and role in disease","volume":"1830","author":"Mar\u00ed","year":"2013","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbadis.2021.166100_bb0295","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1016\/j.tibs.2016.01.001","article-title":"Mitochondrial cristae: where beauty meets functionality","volume":"41","author":"Cogliati","year":"2016","journal-title":"Trends Biochem. Sci."},{"key":"10.1016\/j.bbadis.2021.166100_bb0300","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":"10.1016\/j.bbadis.2021.166100_bb0305","doi-asserted-by":"crossref","first-page":"1311","DOI":"10.1093\/hmg\/ddt521","article-title":"ACAD9, a complex I assembly factor with a moonlighting function in fatty acid oxidation deficiencies","volume":"23","author":"Nouws","year":"2014","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2021.166100_bb0310","doi-asserted-by":"crossref","first-page":"3238","DOI":"10.1093\/hmg\/ddv074","article-title":"Complex I assembly function and fatty acid oxidation enzyme activity of ACAD9 both contribute to disease severity in ACAD9 deficiency","volume":"24","author":"Schiff","year":"2015","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbadis.2021.166100_bb0315","first-page":"333","article-title":"Fasting induces prominent proteomic changes in liver in very long chain Acyl-CoA dehydrogenase deficient mice","volume":"8","author":"Wang","year":"2016","journal-title":"Biochem. Biophys. Rep."},{"key":"10.1016\/j.bbadis.2021.166100_bb0320","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1002\/jcp.25804","article-title":"The role of various peroxisome proliferator-activated receptors and their ligands in clinical practice","volume":"233","author":"Giuseppe","year":"2018","journal-title":"J. Cell. Physiol."},{"key":"10.1016\/j.bbadis.2021.166100_bb0325","doi-asserted-by":"crossref","first-page":"720","DOI":"10.1016\/j.jhep.2014.10.039","article-title":"Molecular mechanism of PPAR\u03b1 action and its impact on lipid metabolism, inflammation and fibrosis in non-alcoholic fatty liver disease","volume":"62","author":"Pawlak","year":"2015","journal-title":"J. Hepatol."},{"key":"10.1016\/j.bbadis.2021.166100_bb0330","doi-asserted-by":"crossref","first-page":"3042","DOI":"10.1093\/ndt\/gfp258","article-title":"Peroxisome proliferator-activated receptor alpha plays a crucial role in l -carnitine anti-apoptosis effect in renal tubular cells","volume":"24","author":"Chen","year":"2009","journal-title":"Nephrol. Dial. Transplant."},{"key":"10.1016\/j.bbadis.2021.166100_bb0335","doi-asserted-by":"crossref","first-page":"305","DOI":"10.1007\/BF00625805","article-title":"Steady-state kinetics of bezafibrate and clofibrate in healthy female volunteers","volume":"17","author":"Abshagen","year":"1980","journal-title":"Eur. J. Clin. Pharmacol."},{"key":"10.1016\/j.bbadis.2021.166100_bb0340","doi-asserted-by":"crossref","first-page":"536","DOI":"10.1136\/ard.51.4.536","article-title":"Bezafibrate induced rhabdomyolysis","volume":"51","author":"Kanterewicz","year":"1992","journal-title":"Ann. Rheum. Dis."},{"key":"10.1016\/j.bbadis.2021.166100_bb0345","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1007\/s13340-012-0104-9","article-title":"Mitochondria toxicity of antihyperlipidemic agents bezafibrate and fenofibrate","volume":"4","author":"Yamada","year":"2013","journal-title":"Diabetol. Int."},{"key":"10.1016\/j.bbadis.2021.166100_bb0350","doi-asserted-by":"crossref","first-page":"659","DOI":"10.1016\/S0891-5849(01)00632-3","article-title":"Mechanism of clofibrate hepatotoxicity: mitochondrial damage and oxidative stress in hepatocytes","volume":"31","author":"Qu","year":"2001","journal-title":"Free Radic. Biol. Med."},{"key":"10.1016\/j.bbadis.2021.166100_bb0355","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pcbi.1003186","article-title":"Biochemical competition makes fatty-acid beta-oxidation vulnerable to substrate overload","volume":"9","author":"van Eunen","year":"2013","journal-title":"PLoS Comput. Biol."},{"key":"10.1016\/j.bbadis.2021.166100_bb0360","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pcbi.1005461","article-title":"The promiscuous enzyme medium-chain 3-keto-acyl-CoA thiolase triggers a vicious cycle in fatty-acid beta-oxidation","volume":"13","author":"Martines","year":"2017","journal-title":"PLoS Comput. Biol."},{"key":"10.1016\/j.bbadis.2021.166100_bb0365","doi-asserted-by":"crossref","first-page":"1652S","DOI":"10.1093\/jn\/136.6.1652S","article-title":"Mammalian cysteine metabolism: new insights into regulation of cysteine metabolism","volume":"136","author":"Stipanuk","year":"2006","journal-title":"J. Nutr."},{"key":"10.1016\/j.bbadis.2021.166100_bb0370","doi-asserted-by":"crossref","first-page":"229","DOI":"10.1016\/S0003-9861(02)00468-X","article-title":"Identification of S-glutathionylated cellular proteins during oxidative stress and constitutive metabolism by affinity purification and proteomic analysis","volume":"406","author":"Lind","year":"2002","journal-title":"Arch. Biochem. Biophys."},{"key":"10.1016\/j.bbadis.2021.166100_bb0375","doi-asserted-by":"crossref","first-page":"231","DOI":"10.1016\/j.freeradbiomed.2008.04.029","article-title":"Fatty acids as modulators of the cellular production of reactive oxygen species","volume":"45","author":"Sch\u00f6nfeld","year":"2008","journal-title":"Free Radic. Biol. Med."},{"key":"10.1016\/j.bbadis.2021.166100_bb0380","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1016\/j.freeradbiomed.2016.05.011","article-title":"Regulation of nadph oxidases in skeletal muscle","volume":"98","author":"Ferreira","year":"2016","journal-title":"Free Radic. Biol. Med."},{"key":"10.1016\/j.bbadis.2021.166100_bb0385","doi-asserted-by":"crossref","first-page":"2022","DOI":"10.1016\/j.bbamem.2009.05.004","article-title":"Cardiolipin acts as a mitochondrial signalling platform to launch apoptosis","volume":"1788","author":"Schug","year":"2009","journal-title":"Biochim. Biophys. Acta Biomembr."},{"key":"10.1016\/j.bbadis.2021.166100_bb0390","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":"10.1016\/j.bbadis.2021.166100_bb0395","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1113\/jphysiol.2003.049478","article-title":"Mitochondrial formation of reactive oxygen species","volume":"552","author":"Turrens","year":"2003","journal-title":"J. Physiol."},{"key":"10.1016\/j.bbadis.2021.166100_bb0400","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1016\/j.exger.2010.01.003","article-title":"The sites and topology of mitochondrial superoxide production","volume":"45","author":"Brand","year":"2010","journal-title":"Exp. Gerontol."},{"key":"10.1016\/j.bbadis.2021.166100_bb0405","doi-asserted-by":"crossref","first-page":"38720","DOI":"10.1074\/jbc.M506575200","article-title":"Acute glutathione depletion restricts mitochondrial ATP export in cerebellar granule neurons","volume":"280","author":"Vesce","year":"2005","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbadis.2021.166100_bb0410","doi-asserted-by":"crossref","first-page":"26","DOI":"10.3390\/nu8010026","article-title":"Peroxisome proliferator-activated receptor activation is associated with altered plasma one-carbon metabolites and B-vitamin status in rats","volume":"8","author":"Lysne","year":"2016","journal-title":"Nutrients"},{"key":"10.1016\/j.bbadis.2021.166100_bb0415","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1016\/j.semcdb.2016.08.003","article-title":"Muscle redox disturbances and oxidative stress as pathomechanisms and therapeutic targets in early-onset myopathies","volume":"64","author":"Moulin","year":"2017","journal-title":"Semin. Cell Dev. Biol."},{"key":"10.1016\/j.bbadis.2021.166100_bb0420","doi-asserted-by":"crossref","first-page":"49","DOI":"10.1016\/j.toxlet.2019.03.001","article-title":"Establishment and characterization of a mouse model of rhabdomyolysis by coadministration of statin and fibrate","volume":"307","author":"Watanabe","year":"2019","journal-title":"Toxicol. Lett."}],"container-title":["Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0925443921000338?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0925443921000338?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,14]],"date-time":"2025-10-14T05:00:21Z","timestamp":1760418021000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0925443921000338"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,6]]},"references-count":84,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2021,6]]}},"alternative-id":["S0925443921000338"],"URL":"https:\/\/doi.org\/10.1016\/j.bbadis.2021.166100","relation":{},"ISSN":["0925-4439"],"issn-type":[{"value":"0925-4439","type":"print"}],"subject":[],"published":{"date-parts":[[2021,6]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Bezafibrate activation of PPAR drives disturbances in mitochondrial redox bioenergetics and decreases the viability of cells from patients with VLCAD deficiency","name":"articletitle","label":"Article Title"},{"value":"Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.bbadis.2021.166100","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2021 The Authors. Published by Elsevier B.V.","name":"copyright","label":"Copyright"}],"article-number":"166100"}}