bezafibrate has been researched along with Lipid Metabolism, Inborn Error in 15 studies
| Timeframe | Studies, this research(%) | All Research% |
|---|---|---|
| pre-1990 | 0 (0.00) | 18.7374 |
| 1990's | 0 (0.00) | 18.2507 |
| 2000's | 3 (20.00) | 29.6817 |
| 2010's | 10 (66.67) | 24.3611 |
| 2020's | 2 (13.33) | 2.80 |
| Authors | Studies |
|---|---|
| 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 |
| Bilet, L; Bosma, M; Havekes, B; Hesselink, MK; Hoeks, J; Janssen, MC; Jorgensen, J; Paglialunga, S; Sauerwein, H; Schaart, G; Schrauwen, P; Schrauwen-Hinderling, VB; Smeets, JL; Sparks, L; van de Weijer, T; Wildberger, J; Zechner, R | 1 |
| Cornelius, N; Gregersen, N; Olsen, RK | 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 |
| Andresen, BS; Bastin, J; Benoist, JF; Boutron, A; de Lonlay, P; Djouadi, F; Ferdinandusse, S; Fukao, T; Habarou, F; Le Bachelier, C; Olpin, S; Schlemmer, D; Strauss, AW; Visser, G; Wanders, RJ; Yamaguchi, S | 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 |
| Bastin, J; Djouadi, F; Gobin-Limballe, S; Kim, JJ; McAndrew, RP | 1 |
| Aubey, F; Bastin, J; Djouadi, F; Ruiter, JP; Schlemmer, D; Strauss, AW; Wanders, RJ | 1 |
| Andresen, BS; Aubey, F; Bastin, J; Djouadi, F; Fukao, T; Gobin-Limballe, S; Kim, JJ; Mandel, H; McAndrew, R; Olpin, S; Ruiter, JP; Wanders, RJ; Yamaguchi, S | 1 |
1 review(s) available for bezafibrate and Lipid Metabolism, Inborn Error
| Article | Year |
|---|---|
Genetic and cellular modifiers of oxidative stress: what can we learn from fatty acid oxidation defects?
Topics: Antioxidants; Apoptosis; Bezafibrate; Electron Transport Chain Complex Proteins; Fatty Acids; Genetic Variation; Genotype; Humans; Lipid Metabolism, Inborn Errors; Necrosis; Oxidative Stress; Phenotype; Protein Folding; Reactive Nitrogen Species; Reactive Oxygen Species | 2013 |
1 trial(s) available for bezafibrate and Lipid Metabolism, Inborn Error
| 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 |
13 other study(ies) available for bezafibrate and Lipid Metabolism, Inborn Error
| Article | Year |
|---|---|
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 |
Effects of bezafibrate treatment in a patient and a carrier with mutations in the PNPLA2 gene, causing neutral lipid storage disease with myopathy.
Topics: Adult; Alleles; Bezafibrate; Fatty Acids; Female; Humans; Hypolipidemic Agents; Lipase; Lipid Metabolism; Lipid Metabolism, Inborn Errors; Muscular Diseases; Mutation; Treatment Outcome | 2013 |
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 |
Mitochondrial trifunctional protein deficiency in human cultured fibroblasts: effects of bezafibrate.
Topics: Bezafibrate; Cardiomyopathies; Cell Line; Fibroblasts; Genotype; Humans; Hypolipidemic Agents; Lipid Metabolism, Inborn Errors; Mitochondrial Myopathies; Mitochondrial Trifunctional Protein; Mitochondrial Trifunctional Protein, alpha Subunit; Mitochondrial Trifunctional Protein, beta Subunit; Mutation; Nervous System Diseases; Rhabdomyolysis | 2016 |
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 |
Compared effects of missense mutations in Very-Long-Chain Acyl-CoA Dehydrogenase deficiency: Combined analysis by structural, functional and pharmacological approaches.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Adult; Amino Acid Substitution; Bezafibrate; Blotting, Western; Case-Control Studies; Fatty Acids; Fibroblasts; Humans; Lipid Metabolism, Inborn Errors; Mutation, Missense; Protein Conformation; Skin; Structure-Activity Relationship | 2010 |
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.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Bezafibrate; Blotting, Western; Carnitine; DNA Primers; Dose-Response Relationship, Drug; Fibroblasts; Gene Expression Regulation, Enzymologic; Humans; Lipid Metabolism, Inborn Errors; Mitochondria; Mutation, Missense; Palmitates; Peroxisome Proliferator-Activated Receptors; Reverse Transcriptase Polymerase Chain Reaction; RNA, Messenger | 2005 |
Genetic basis for correction of very-long-chain acyl-coenzyme A dehydrogenase deficiency by bezafibrate in patient fibroblasts: toward a genotype-based therapy.
Topics: Acyl-CoA Dehydrogenase, Long-Chain; Animals; Bezafibrate; Cells, Cultured; Fatty Acids; Fibroblasts; Genetic Therapy; Genotype; Humans; Hypolipidemic Agents; Lipid Metabolism, Inborn Errors; Models, Molecular; Polymerase Chain Reaction; Rats; RNA, Messenger; Skin | 2007 |