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idebenone and Electron Transport Chain Deficiencies, Mitochondrial

idebenone has been researched along with Electron Transport Chain Deficiencies, Mitochondrial in 14 studies

Research Excerpts

ExcerptRelevanceReference
" CoQ10 along with its synthetic analogue, idebenone, is used largely to treat various neurodegenerative diseases including Alzheimer's disease, Parkinson's disease, Huntington's disease, Amyotrophic lateral sclerosis, and Friedreich's ataxia and additional brain disease condition like autism, multiple sclerosis, epilepsy, depression, and bipolar disorder, which are related to mitochondrial impairment."9.12Coenzyme Q10 a mitochondrial restorer for various brain disorders. ( Pradhan, N; Singh, A; Singh, C, 2021)
" CoQ10 along with its synthetic analogue, idebenone, is used largely to treat various neurodegenerative diseases including Alzheimer's disease, Parkinson's disease, Huntington's disease, Amyotrophic lateral sclerosis, and Friedreich's ataxia and additional brain disease condition like autism, multiple sclerosis, epilepsy, depression, and bipolar disorder, which are related to mitochondrial impairment."5.12Coenzyme Q10 a mitochondrial restorer for various brain disorders. ( Pradhan, N; Singh, A; Singh, C, 2021)
" Idebenone treatment significantly corrected cardiac diastolic dysfunction and prevented mortality from cardiac pump failure induced by dobutamine stress testing in vivo, significantly reduced cardiac inflammation and fibrosis, and significantly improved voluntary running performance in mdx mice."3.75Long-term blinded placebo-controlled study of SNT-MC17/idebenone in the dystrophin deficient mdx mouse: cardiac protection and improved exercise performance. ( Barzaghi, P; Buyse, GM; Courdier-Fruh, I; D'hooge, J; Erb, M; Herijgers, P; Jara, A; Meier, T; Mertens, L; Van Den Bergh, A; Van der Mieren, G; Verbeken, E, 2009)
"Mitochondrial diseases are a group of devastating disorders for which there is no transformative cure."1.48Patient-derived lymphoblastoid cell lines harboring mitochondrial DNA mutations as tool for small molecule drug discovery. ( Brown, JM; Chin, RM; Johnson, KK; Panavas, T, 2018)
"Idebenone is a hydrophilic short-chain coenzyme (Co) Q analogue, which has been used as a potential bypass of defective complex I in both Leber Hereditary Optic Neuropathy and OPA1-dependent Dominant Optic Atrophy."1.48The idebenone metabolite QS10 restores electron transfer in complex I and coenzyme Q defects. ( Acosta Lopez, MJ; Argenton, F; Bernardi, P; Carelli, V; Carini, M; Da Ros, T; Galber, C; Giorgio, V; Petronilli, V; Prato, M; Salviati, L; Schiavone, M, 2018)

Research

Studies (14)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's1 (7.14)29.6817
2010's11 (78.57)24.3611
2020's2 (14.29)2.80

Authors

AuthorsStudies
Pradhan, N1
Singh, C1
Singh, A2
Faccenda, D1
Campanella, M1
Bianco, A1
Bisceglia, L1
Russo, L1
Palese, LL1
D'Agruma, L1
Emperador, S1
Montoya, J1
Guerriero, S1
Petruzzella, V1
Chin, RM1
Panavas, T1
Brown, JM1
Johnson, KK1
Giorgio, V1
Schiavone, M1
Galber, C1
Carini, M1
Da Ros, T1
Petronilli, V1
Argenton, F1
Carelli, V1
Acosta Lopez, MJ1
Salviati, L1
Prato, M1
Bernardi, P1
Kleiner, G1
Barca, E1
Ziosi, M1
Emmanuele, V1
Xu, Y1
Hidalgo-Gutierrez, A1
Qiao, C1
Tadesse, S1
Area-Gomez, E1
Lopez, LC1
Quinzii, CM1
Liu, J1
Wang, LN1
Hargreaves, IP1
Buyse, GM1
Van der Mieren, G1
Erb, M2
D'hooge, J1
Herijgers, P1
Verbeken, E1
Jara, A1
Van Den Bergh, A1
Mertens, L1
Courdier-Fruh, I1
Barzaghi, P1
Meier, T1
Mancuso, M1
Orsucci, D1
Volpi, L1
Calsolaro, V1
Siciliano, G1
Kerr, DS2
Koopman, WJ1
Willems, PH1
Smeitink, JA1
Hoffmann-Enger, B1
Deppe, H1
Soeberdt, M1
Haefeli, RH1
Rummey, C1
Feurer, A1
Gueven, N1

Reviews

8 reviews available for idebenone and Electron Transport Chain Deficiencies, Mitochondrial

ArticleYear
Coenzyme Q10 a mitochondrial restorer for various brain disorders.
    Naunyn-Schmiedeberg's archives of pharmacology, 2021, Volume: 394, Issue:11

    Topics: Animals; Antioxidants; Brain Diseases; Humans; Mitochondria; Mitochondrial Diseases; Neurodegenerati

2021
Pharmacological advances in mitochondrial therapy.
    EBioMedicine, 2021, Volume: 65

    Topics: Antioxidants; Humans; Mitochondria; Mitochondrial Diseases; Mitochondrial Dynamics; Neurodegenerativ

2021
Mitochondrial enhancement for neurodegenerative movement disorders: a systematic review of trials involving creatine, coenzyme Q10, idebenone and mitoquinone.
    CNS drugs, 2014, Volume: 28, Issue:1

    Topics: Animals; Creatine; Dose-Response Relationship, Drug; Humans; Mitochondria; Mitochondrial Diseases; N

2014
Coenzyme Q10 as a therapy for mitochondrial disease.
    The international journal of biochemistry & cell biology, 2014, Volume: 49

    Topics: Animals; Ataxia; Humans; Mitochondrial Diseases; Molecular Structure; Muscle Weakness; Treatment Out

2014
Coenzyme Q10 in neuromuscular and neurodegenerative disorders.
    Current drug targets, 2010, Volume: 11, Issue:1

    Topics: Animals; Humans; Mitochondrial Diseases; Neurodegenerative Diseases; Neuromuscular Diseases; Ubiquin

2010
Treatment of mitochondrial electron transport chain disorders: a review of clinical trials over the past decade.
    Molecular genetics and metabolism, 2010, Volume: 99, Issue:3

    Topics: Arginine; Clinical Trials as Topic; Dichloroacetic Acid; Humans; Mitochondrial Diseases; Randomized

2010
Monogenic mitochondrial disorders.
    The New England journal of medicine, 2012, Mar-22, Volume: 366, Issue:12

    Topics: Adenosine Triphosphate; Cyclosporine; Enzyme Replacement Therapy; Humans; Mitochondria; Mitochondria

2012
Review of clinical trials for mitochondrial disorders: 1997-2012.
    Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics, 2013, Volume: 10, Issue:2

    Topics: Antioxidants; Arginine; Clinical Trials as Topic; Creatine; Exercise Therapy; Humans; Mitochondrial

2013

Other Studies

6 other studies available for idebenone and Electron Transport Chain Deficiencies, Mitochondrial

ArticleYear
High Mitochondrial DNA Copy Number Is a Protective Factor From Vision Loss in Heteroplasmic Leber's Hereditary Optic Neuropathy (LHON).
    Investigative ophthalmology & visual science, 2017, Apr-01, Volume: 58, Issue:4

    Topics: Antioxidants; Blindness; DNA Copy Number Variations; DNA, Mitochondrial; Female; Genes, Mitochondria

2017
Patient-derived lymphoblastoid cell lines harboring mitochondrial DNA mutations as tool for small molecule drug discovery.
    BMC research notes, 2018, Mar-27, Volume: 11, Issue:1

    Topics: Adult; Cell Line; Child; Child, Preschool; DNA, Mitochondrial; Drug Discovery; Female; Humans; Lymph

2018
The idebenone metabolite QS10 restores electron transfer in complex I and coenzyme Q defects.
    Biochimica et biophysica acta. Bioenergetics, 2018, Volume: 1859, Issue:9

    Topics: Adenosine Triphosphate; Animals; Antioxidants; Ataxia; Cell Respiration; Cells, Cultured; Electron T

2018
CoQ
    Biochimica et biophysica acta. Molecular basis of disease, 2018, Volume: 1864, Issue:11

    Topics: Alkyl and Aryl Transferases; Animals; Antioxidants; Ataxia; Disease Models, Animal; HeLa Cells; Huma

2018
Long-term blinded placebo-controlled study of SNT-MC17/idebenone in the dystrophin deficient mdx mouse: cardiac protection and improved exercise performance.
    European heart journal, 2009, Volume: 30, Issue:1

    Topics: Animals; Antioxidants; Biomarkers; Cardiotonic Agents; Diastole; Dobutamine; Echocardiography; Fibro

2009
Features of idebenone and related short-chain quinones that rescue ATP levels under conditions of impaired mitochondrial complex I.
    PloS one, 2012, Volume: 7, Issue:4

    Topics: Adenosine Triphosphate; Animals; Antioxidants; Cell Line; Chemical Phenomena; Electron Transport Com

2012