bezafibrate has been researched along with Mitochondrial Diseases in 17 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 4 (23.53) | 29.6817 |
| 2010's | 9 (52.94) | 24.3611 |
| 2020's | 4 (23.53) | 2.80 |
| Authors | Studies |
|---|---|
| Chen, Y; Mu, T; Shao, Q; Tong, N; Yang, X; Zhang, X; Zhang, Y; Zhou, Y | 1 |
| de Haas, R; Frambach, SJCM; Russel, FGM; Schirris, TJJ; Smeitink, JAM; van de Wal, MAE; van den Broek, PHH | 1 |
| Asahina, N; Egawa, K; Hayashi, H; Ishige, M; Isoe, T; Kawakami, S; Kobayashi, K; Kuzume, K; Miyakoshi, T; Nakamagoe, K; Oba, K; Ochi, F; Sameshima, K; Sato, N; Shiraishi, H; Tamaoka, A; Watanabe, A; Watanabe, K; Yamada, K; Yamaguchi, S; Yokoshiki, S | 1 |
| Andersen, KG; Gregersen, N; Hargreaves, IP; Heaton, R; Lund, M; Olsen, RKJ | 1 |
| Komen, JC; Thorburn, DR | 1 |
| Andersen, G; Laforêt, P; Madsen, KL; Preisler, N; Vissing, J; Ørngreen, MC | 1 |
| Bastin, J; Bonnefont, JP; Bresson, JL; Djouadi, F | 1 |
| Laforét, P; Vissing, J; Ørngreen, MC | 1 |
| Aoyama, Y; Densupsoontorn, N; Fukao, T; Jirapinyo, P; Sathienkijkanchai, A; Vatanavicharn, N; Wasant, P; Yamada, K; Yamaguchi, S | 1 |
| Ding, Y; Li, X; Liu, Y; Ma, Y; Song, J; Wang, Q; Yang, Y | 1 |
| Harrington, M | 1 |
| Bastin, J; Behin, A; Bonnefont, JP; Djouadi, F | 1 |
| Fukuda, S; Hasegawa, Y; Kobayashi, H; Li, H; Mushimoto, Y; Purevsuren, J; Yamaguchi, S | 1 |
| Marini, M; Moraes, CT; Wang, X; Wenz, T | 1 |
| Fukao, T; Fukuda, S; Furui, M; Hasegawa, Y; Kobayashi, H; Li, H; Mushimoto, Y; Purevsuren, J; Takahashi, T; Taketani, T; Yamada, K; Yamaguchi, S | 1 |
| Bottoni, P; Castagnola, M; De Sole, P; Giardina, B; Maggiano, N; Martorana, GE; Messana, I; Nocca, G; Scatena, R; Vincenzoni, F | 1 |
| Aubey, F; Bastin, J; Djouadi, F; Munnich, A; Rötig, A | 1 |
1 review(s) available for bezafibrate and Mitochondrial Diseases
| Article | Year |
|---|---|
Turn up the power - pharmacological activation of mitochondrial biogenesis in mouse models.
Topics: Aminoimidazole Carboxamide; AMP-Activated Protein Kinases; Animals; Bezafibrate; Disease Models, Animal; Energy Metabolism; Mitochondria; Mitochondrial Diseases; Mitochondrial Turnover; Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; Resveratrol; Ribonucleotides; Sirtuin 1; Stilbenes; Transcription Factors; Up-Regulation | 2014 |
1 trial(s) available for bezafibrate and Mitochondrial Diseases
| Article | Year |
|---|---|
Bezafibrate in skeletal muscle fatty acid oxidation disorders: a randomized clinical trial.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adolescent; Adult; Aged; Bezafibrate; Carnitine O-Palmitoyltransferase; Clinical Protocols; Congenital Bone Marrow Failure Syndromes; Cross-Over Studies; Fatty Acids; Female; Humans; Hypolipidemic Agents; Lipid Metabolism, Inborn Errors; Male; Middle Aged; Mitochondrial Diseases; Muscle, Skeletal; Muscular Diseases; Treatment Outcome; Young Adult | 2014 |
15 other study(ies) available for bezafibrate and Mitochondrial Diseases
| Article | Year |
|---|---|
Maternally inherited diabetes and deafness coexists with lipoprotein lipase gene mutation-associated severe hyperlipidemia that was resistant to fenofibrate and atorvastatin, but sensitive to bezafibrate: A case report.
Topics: Atorvastatin; Bezafibrate; Deafness; Diabetes Mellitus, Type 2; Female; Fenofibrate; Humans; Hyperlipidemias; Lipoprotein Lipase; Mitochondrial Diseases; Mutation | 2022 |
Effects of clofibrate and KH176 on life span and motor function in mitochondrial complex I-deficient mice.
Topics: Adenosine Triphosphate; Animals; Bezafibrate; Chromans; Clofibrate; Electron Transport Complex I; Fatty Acids; Humans; Leigh Disease; Longevity; Mice; Mice, Knockout; Mitochondria; Mitochondrial Diseases; Motor Activity; Oxidation-Reduction; Peroxisome Proliferator-Activated Receptors | 2020 |
Efficacy of bezafibrate for preventing myopathic attacks in patients with very long-chain acyl-CoA dehydrogenase deficiency.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adult; Bezafibrate; Child; Congenital Bone Marrow Failure Syndromes; Female; Humans; Lipid Metabolism, Inborn Errors; Male; Mitochondrial Diseases; Muscular Diseases | 2021 |
Bezafibrate activation of PPAR drives disturbances in mitochondrial redox bioenergetics and decreases the viability of cells from patients with VLCAD deficiency.
Topics: Bezafibrate; Congenital Bone Marrow Failure Syndromes; Energy Metabolism; Fibroblasts; Humans; Hypolipidemic Agents; Lipid Metabolism, Inborn Errors; Mitochondria; Mitochondrial Diseases; Muscular Diseases; Oxidative Stress; Peroxisome Proliferator-Activated Receptors | 2021 |
Should the beneficial impact of bezafibrate on fatty acid oxidation disorders be questioned?
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Bezafibrate; Carnitine O-Palmitoyltransferase; Congenital Bone Marrow Failure Syndromes; Fatty Acids; Heart Rate; Humans; Lipid Metabolism, Inborn Errors; Lipolysis; Metabolism, Inborn Errors; Mitochondrial Diseases; Muscular Diseases; Oxidation-Reduction; Treatment Outcome | 2015 |
No effect of bezafibrate in patients with CPTII and VLCAD deficiencies.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Bezafibrate; Carnitine O-Palmitoyltransferase; Congenital Bone Marrow Failure Syndromes; Fatty Acids; Humans; Lipid Metabolism, Inborn Errors; Lipolysis; Metabolism, Inborn Errors; Mitochondrial Diseases; Muscular Diseases | 2015 |
Carnitine-acylcarnitine translocase deficiency: Two neonatal cases with common splicing mutation and in vitro bezafibrate response.
Topics: Bezafibrate; Carnitine; Carnitine Acyltransferases; Cells, Cultured; Child, Preschool; Fatal Outcome; Female; Fibroblasts; Genes, Lethal; Humans; Hypolipidemic Agents; In Vitro Techniques; Infant; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Membrane Transport Proteins; Mitochondrial Diseases; Mutation; Treatment Outcome | 2015 |
Very long-chain acyl-coenzyme A dehydrogenase deficiency in Chinese patients: eight case reports, including one case of prenatal diagnosis.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Acyl-CoA Dehydrogenases; Amniotic Fluid; Ascorbic Acid; Asian People; Bezafibrate; Carnitine; Case-Control Studies; China; Chromatography, Liquid; Congenital Bone Marrow Failure Syndromes; DNA, Complementary; Exons; Female; Genetic Testing; Heterozygote; Humans; Infant; Infant Formula; Infant, Newborn; Lipid Metabolism, Inborn Errors; Male; Mitochondrial Diseases; Muscular Diseases; Mutation, Missense; Neonatal Screening; Prenatal Diagnosis; Sequence Alignment; Sequence Analysis, DNA; Tandem Mass Spectrometry; Treatment Outcome; Triglycerides; Vitamin B Complex | 2015 |
Mice, mitochondria and myopathy.
Topics: Animals; Bezafibrate; Female; Mice; Mice, Knockout; Mitochondria; Mitochondrial Diseases; Muscular Diseases; Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; Trans-Activators; Transcription Factors | 2008 |
Bezafibrate for an inborn mitochondrial beta-oxidation defect.
Topics: Adult; Bezafibrate; Carnitine O-Palmitoyltransferase; Genes, Recessive; Humans; Hypolipidemic Agents; Lipid Metabolism, Inborn Errors; Mitochondrial Diseases; Oxidation-Reduction; Palmitoylcarnitine; Pilot Projects; Quality of Life; Rhabdomyolysis; RNA, Messenger; Statistics, Nonparametric | 2009 |
Effect of heat stress and bezafibrate on mitochondrial beta-oxidation: comparison between cultured cells from normal and mitochondrial fatty acid oxidation disorder children using in vitro probe acylcarnitine profiling assay.
Topics: Acyl-CoA Dehydrogenase; Acyl-CoA Dehydrogenase, Long-Chain; Bezafibrate; Carnitine; Cells, Cultured; Child; Fatty Acids; Fibroblasts; Hot Temperature; Humans; Hypolipidemic Agents; Lipid Metabolism, Inborn Errors; Mitochondria; Mitochondrial Diseases; Oxidation-Reduction; Stress, Physiological | 2010 |
A metabolic shift induced by a PPAR panagonist markedly reduces the effects of pathogenic mitochondrial tRNA mutations.
Topics: Adenosine Triphosphate; Bezafibrate; Cell Line; DNA, Mitochondrial; Humans; Mitochondria; Mitochondrial Diseases; Mutation; Oxidative Phosphorylation; Peroxisome Proliferator-Activated Receptors; PPAR gamma; Protein Biosynthesis; RNA; RNA, Mitochondrial; RNA, Transfer | 2011 |
Bezafibrate can be a new treatment option for mitochondrial fatty acid oxidation disorders: evaluation by in vitro probe acylcarnitine assay.
Topics: Bezafibrate; Carboxylic Acids; Carnitine; Cells, Cultured; Fatty Acids; Fibroblasts; Humans; Mitochondrial Diseases; Oxidation-Reduction | 2012 |
Bezafibrate induces a mitochondrial derangement in human cell lines: a PPAR-independent mechanism for a peroxisome proliferator.
Topics: Acetates; Alanine; Animals; Bezafibrate; Dose-Response Relationship, Drug; Humans; Hypolipidemic Agents; Italy; Lactic Acid; Magnetic Resonance Spectroscopy; Microscopy, Electron; Mitochondria; Mitochondrial Diseases; Peroxisome Proliferators; Rats; Time Factors; Tumor Cells, Cultured | 2003 |
Activation of peroxisome proliferator-activated receptor pathway stimulates the mitochondrial respiratory chain and can correct deficiencies in patients' cells lacking its components.
Topics: Bezafibrate; Cells, Cultured; Electron Transport; Electron Transport Complex I; Electron Transport Complex III; Electron Transport Complex IV; Gene Expression Regulation; Heat-Shock Proteins; Humans; Mitochondrial Diseases; Peroxisome Proliferator-Activated Receptor Gamma Coactivator 1-alpha; Peroxisome Proliferator-Activated Receptors; Transcription Factors | 2008 |