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6-hydroxy-2,5,7,8-tetramethylchroman-2-carboxylic acid and Electron Transport Chain Deficiencies, Mitochondrial

6-hydroxy-2,5,7,8-tetramethylchroman-2-carboxylic acid has been researched along with Electron Transport Chain Deficiencies, Mitochondrial in 8 studies

Research

Studies (8)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's1 (12.50)29.6817
2010's5 (62.50)24.3611
2020's2 (25.00)2.80

Authors

AuthorsStudies
de Haas, R; Frambach, SJCM; Russel, FGM; Schirris, TJJ; Smeitink, JAM; van de Wal, MAE; van den Broek, PHH1
Beyrath, J; Hankemeier, T; Jiang, X; Pecheritsyna, S; Pennings, B; Renkema, H; Schoeman, JC; Smeitink, J1
Beyrath, J; Collin, RWJ; Das, D; de Haas, R; Garanto, A; Greupink, R; Heerschap, A; Pertijs, J; Renkema, HG; Russel, FG; Smeitink, JA; van den Broek, P; Willems, P1
Bekel, A; Beyrath, J; Eftekhari, P; Houben, L; Pecheritsyna, S; Pellegrini, M; Renkema, H; Smeitink, JAM; van den Broek, P; van Zandvoort, P1
Beukema, R; Beyrath, J; de Laat, P; Groothuis, J; Hemelaar, P; Janssen, MCH; Koene, S; Pickkers, P; Smeitink, J; Spaans, E; Verhaak, C1
Beyrath, J; Blanchet, L; Buydens, LM; Jonckheere, AI; Koopman, WJ; Pellegrini, M; Rodenburg, RJ; Smeitink, JA; van Emst-de Vries, SE; Vogels, C; Willems, PH1
Distelmaier, F; Koopman, WJ; Mayatepek, E; Smeitink, JA; Visch, HJ; Willems, PH1
Dieteren, CE; Koopman, WJ; Nijtmans, LG; Roestenberg, P; Smeitink, JA; Valsecchi, F; Willems, PH1

Reviews

1 review(s) available for 6-hydroxy-2,5,7,8-tetramethylchroman-2-carboxylic acid and Electron Transport Chain Deficiencies, Mitochondrial

ArticleYear
Mammalian mitochondrial complex I: biogenesis, regulation, and reactive oxygen species generation.
    Antioxidants & redox signaling, 2010, Jun-15, Volume: 12, Issue:12

    Topics: Animals; Biological Transport; Cattle; Chromans; Electron Transport; Electron Transport Complex I; Eukaryotic Cells; Fibroblasts; Humans; Mammals; Membrane Lipids; Mitochondria; Mitochondrial Diseases; Mitochondrial Proteins; NADH Dehydrogenase; Organ Specificity; Oxidative Phosphorylation; Reactive Oxygen Species; Rotenone; Signal Transduction

2010

Trials

1 trial(s) available for 6-hydroxy-2,5,7,8-tetramethylchroman-2-carboxylic acid and Electron Transport Chain Deficiencies, Mitochondrial

ArticleYear
The KHENERGY Study: Safety and Efficacy of KH176 in Mitochondrial m.3243A>G Spectrum Disorders.
    Clinical pharmacology and therapeutics, 2019, Volume: 105, Issue:1

    Topics: Administration, Oral; Adult; Antioxidants; Chromans; Cross-Over Studies; DNA, Mitochondrial; Double-Blind Method; Female; Humans; Male; Middle Aged; Mitochondrial Diseases; Mutation; Treatment Outcome

2019

Other Studies

6 other study(ies) available for 6-hydroxy-2,5,7,8-tetramethylchroman-2-carboxylic acid and Electron Transport Chain Deficiencies, Mitochondrial

ArticleYear
Effects of clofibrate and KH176 on life span and motor function in mitochondrial complex I-deficient mice.
    Biochimica et biophysica acta. Molecular basis of disease, 2020, 06-01, Volume: 1866, Issue:6

    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
Mechanism of action and potential applications of selective inhibition of microsomal prostaglandin E synthase-1-mediated PGE
    Scientific reports, 2021, 01-13, Volume: 11, Issue:1

    Topics: Animals; Cells, Cultured; Chromans; Cyclooxygenase 2; Dinoprostone; Fibroblasts; Humans; Inflammation; Interleukin-1beta; Intramolecular Oxidoreductases; Lipopolysaccharides; Mice; Microsomes; Mitochondrial Diseases; Oxidation-Reduction; Prostaglandin-E Synthases; RAW 264.7 Cells

2021
Therapeutic effects of the mitochondrial ROS-redox modulator KH176 in a mammalian model of Leigh Disease.
    Scientific reports, 2017, 09-15, Volume: 7, Issue:1

    Topics: Animals; Brain; Chromans; Diffusion Tensor Imaging; Electron Transport Complex I; Gait; Leigh Disease; Mice; Mice, Knockout; Mitochondrial Diseases; Neuroimaging; Reactive Oxygen Species

2017
KH176 Safeguards Mitochondrial Diseased Cells from Redox Stress-Induced Cell Death by Interacting with the Thioredoxin System/Peroxiredoxin Enzyme Machinery.
    Scientific reports, 2018, 04-26, Volume: 8, Issue:1

    Topics: Apoptosis; Cell Death; Cell Line; Chromans; Dose-Response Relationship, Drug; Electron Transport Complex I; Glutathione; Humans; Mitochondria; Mitochondrial Diseases; Models, Molecular; Molecular Conformation; Molecular Structure; Oxidation-Reduction; Oxidative Phosphorylation; Oxidative Stress; Peroxiredoxins; Reactive Oxygen Species; Structure-Activity Relationship; Thioredoxins

2018
Quantifying small molecule phenotypic effects using mitochondrial morpho-functional fingerprinting and machine learning.
    Scientific reports, 2015, Jan-26, Volume: 5

    Topics: Chromans; Citrate (si)-Synthase; Electron Transport Complex I; Fibroblasts; Humans; Leigh Disease; Machine Learning; Membrane Potential, Mitochondrial; Mitochondria; Mitochondrial Diseases; Oxidative Phosphorylation; Reactive Oxygen Species

2015
The antioxidant Trolox restores mitochondrial membrane potential and Ca2+ -stimulated ATP production in human complex I deficiency.
    Journal of molecular medicine (Berlin, Germany), 2009, Volume: 87, Issue:5

    Topics: Adenosine Triphosphate; Antioxidants; Bradykinin; Calcium; Cell Line; Chromans; Dose-Response Relationship, Drug; Electron Transport Complex I; Endoplasmic Reticulum; Fibroblasts; Humans; Membrane Potential, Mitochondrial; Mitochondrial Diseases; Reactive Oxygen Species

2009