Bezafibrate activation of PPAR drives disturbances in mitochondrial redox bioenergetics and decreases the viability of cells from patients with VLCAD deficiency
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Title
Bezafibrate activation of PPAR drives disturbances in mitochondrial redox bioenergetics and decreases the viability of cells from patients with VLCAD deficiency
Authors
Keywords
Mitochondria, Glutathione, Thiols, Bezafibrate, PPAR, Oxidative stress, ROS, VLCAD, VLCAD deficiency, Inborn errors of metabolism, Fatty acids oxidation deficiency
Journal
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
Volume 1867, Issue 6, Pages 166100
Publisher
Elsevier BV
Online
2021-02-06
DOI
10.1016/j.bbadis.2021.166100
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Note: Only part of the references are listed.- Establishment and characterization of a mouse model of rhabdomyolysis by coadministration of statin and fibrate
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- (2017) Anne-Claire M. F. Martines et al. PLoS Computational Biology
- The Biochemistry and Physiology of Mitochondrial Fatty Acid β-Oxidation and Its Genetic Disorders
- (2016) Sander M. Houten et al. Annual Review of Physiology
- Mitochondrial fission and fusion
- (2016) H. Lee et al. BIOCHEMICAL SOCIETY TRANSACTIONS
- Living on the edge: substrate competition explains loss of robustness in mitochondrial fatty-acid oxidation disorders
- (2016) Karen van Eunen et al. BMC BIOLOGY
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- (2016) E. F. Diekman et al. PLoS One
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- (2016) Yang Yang et al. TOHOKU JOURNAL OF EXPERIMENTAL MEDICINE
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- (2016) Sara Cogliati et al. TRENDS IN BIOCHEMICAL SCIENCES
- Peroxisome Proliferator-Activated Receptor Activation is Associated with Altered Plasma One-Carbon Metabolites and B-Vitamin Status in Rats
- (2016) Vegard Lysne et al. Nutrients
- Mitochondrial dysfunction in fatty acid oxidation disorders: insights from human and animal studies
- (2015) M. Wajner et al. BIOSCIENCE REPORTS
- Fatty acid oxidation flux predicts the clinical severity of VLCAD deficiency
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- Bezafibrate in skeletal muscle fatty acid oxidation disorders: A randomized clinical trial
- (2014) M. C. Orngreen et al. NEUROLOGY
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- Myocardial energy shortage and unmet anaplerotic needs in the fasted long-chain acyl-CoA dehydrogenase knockout mouse
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- Comparison of three thiol probes for determination of apoptosis-related changes in cellular redox status
- (2013) Mette E. Skindersoe et al. CYTOMETRY PART A
- Impaired amino acid metabolism contributes to fasting-induced hypoglycemia in fatty acid oxidation defects
- (2013) Sander M. Houten et al. HUMAN MOLECULAR GENETICS
- ACAD9, a complex I assembly factor with a moonlighting function in fatty acid oxidation deficiencies
- (2013) Jessica Nouws et al. HUMAN MOLECULAR GENETICS
- Genetic and cellular modifiers of oxidative stress: What can we learn from fatty acid oxidation defects?
- (2013) Rikke Katrine Jentoft Olsen et al. MOLECULAR GENETICS AND METABOLISM
- Biochemical Competition Makes Fatty-Acid β-Oxidation Vulnerable to Substrate Overload
- (2013) Karen van Eunen et al. PLoS Computational Biology
- Mitochondrial glutathione: Features, regulation and role in disease
- (2012) Montserrat Marí et al. BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS
- A novel and rapid apoptosis assay based on thiol redox status
- (2012) Mette E. Skindersoe et al. CYTOMETRY PART A
- Tissue-Specific Strategies of the Very-Long Chain Acyl-CoA Dehydrogenase-Deficient (VLCAD−/−) Mouse to Compensate a Defective Fatty Acid β-Oxidation
- (2012) Sara Tucci et al. PLoS One
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- Fasting-induced oxidative stress in very long chain acyl-CoA dehydrogenase-deficient mice
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- The use of total protein stains as loading controls: An alternative to high-abundance single-protein controls in semi-quantitative immunoblotting
- (2008) Georgina M. Aldridge et al. JOURNAL OF NEUROSCIENCE METHODS
- Decreased Ubiquinone Availability and Impaired Mitochondrial Cytochrome Oxidase Activity Associated With Statin Treatment
- (2008) Andrew J. Duncan et al. TOXICOLOGY MECHANISMS AND METHODS
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