Page last updated: 2024-10-17

creatine and Electron Transport Chain Deficiencies, Mitochondrial

creatine has been researched along with Electron Transport Chain Deficiencies, Mitochondrial in 23 studies

Research Excerpts

ExcerptRelevanceReference
"Recent investigations have suggested creatine (Cr) as an additional biomarker of mitochondrial diseases."7.79Role of creatine as biomarker of mitochondrial diseases. ( Arias, A; Briones, P; García-Villoria, J; Pajares, S; Ribes, A, 2013)
"Creatine is a molecule that is produced both endogenously, and acquired exogenously through diet, and is an extremely important molecule that participates in buffering intracellular energy stores."6.44Creatine and its potential therapeutic value for targeting cellular energy impairment in neurodegenerative diseases. ( Adhihetty, PJ; Beal, MF, 2008)
"Semiquantitative analysis of NAA relative to creatine-containing compounds (Cr) and choline (Cho) was carried out from proton spectra obtained by means of chemical shift (CS) imaging and single-voxel (SV) methods in 25 patients with severe traumatic brain injuries (TBIs) (Glasgow Coma Scale scores < or = 8) using a 1."5.13Assessment of mitochondrial impairment in traumatic brain injury using high-resolution proton magnetic resonance spectroscopy. ( Aygok, GA; Bullock, RM; Fatouros, PP; Marmarou, A; Portella, G; Signoretti, S, 2008)
"Recent investigations have suggested creatine (Cr) as an additional biomarker of mitochondrial diseases."3.79Role of creatine as biomarker of mitochondrial diseases. ( Arias, A; Briones, P; García-Villoria, J; Pajares, S; Ribes, A, 2013)
"Studies using the mdx model of Duchenne muscular dystrophy have found evidence of enhanced mitochondrial function, reduced intra-cellular calcium and improved performance with CrM supplementation."2.44Clinical use of creatine in neuromuscular and neurometabolic disorders. ( Tarnopolsky, MA, 2007)
"Creatine is a molecule that is produced both endogenously, and acquired exogenously through diet, and is an extremely important molecule that participates in buffering intracellular energy stores."2.44Creatine and its potential therapeutic value for targeting cellular energy impairment in neurodegenerative diseases. ( Adhihetty, PJ; Beal, MF, 2008)
"Patients with mitochondrial disorders offer a unique window through which we can begin to understand the association between psychiatric symptoms and mitochondrial dysfunction in vivo."1.38Psychiatric symptoms correlate with metabolic indices in the hippocampus and cingulate in patients with mitochondrial disorders. ( Anglin, RE; Mazurek, MF; Noseworthy, MD; Rosebush, PI; Tarnopolsky, M, 2012)
"Recently, the clinical spectrum of dominant optic atrophy has been extended to frequent syndromic forms, exhibiting various degrees of neurological and muscle impairments frequently found in mitochondrial diseases."1.38The human OPA1delTTAG mutation induces premature age-related systemic neurodegeneration in mouse. ( Angebault, C; Bielicki, G; Boddaert, N; Brabet, P; Cazevieille, C; Chaix, B; Delettre, C; Gueguen, N; Hamel, CP; Lenaers, G; Mausset-Bonnefont, AL; Puel, JL; Renou, JP; Reynier, P; Rigau, V; Sarzi, E; Seveno, M; Wang, J, 2012)

Research

Studies (23)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's9 (39.13)29.6817
2010's14 (60.87)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Ostojic, SM1
Liu, J1
Wang, LN1
Reid, CA1
Mullen, S1
Kim, TH1
Petrou, S1
Lunsing, RJ1
Strating, K1
de Koning, TJ1
Sijens, PE1
DeBrosse, C1
Nanga, RPR1
Wilson, N1
D'Aquilla, K1
Elliott, M1
Hariharan, H1
Yan, F1
Wade, K1
Nguyen, S1
Worsley, D1
Parris-Skeete, C1
McCormick, E1
Xiao, R1
Cunningham, ZZ1
Fishbein, L1
Nathanson, KL1
Lynch, DR1
Stallings, VA1
Yudkoff, M1
Falk, MJ1
Reddy, R1
McCormack, SE1
Tarnopolsky, MA2
Adhihetty, PJ1
Beal, MF3
Orsucci, D1
Filosto, M1
Siciliano, G1
Mancuso, M1
Shaham, O1
Slate, NG1
Goldberger, O1
Xu, Q1
Ramanathan, A1
Souza, AL1
Clish, CB1
Sims, KB1
Mootha, VK1
Hathaway, SC1
Friez, M1
Limbo, K1
Parker, C1
Salomons, GS1
Vockley, J1
Wood, T1
Abdul-Rahman, OA1
Naia, L1
Ribeiro, MJ1
Rego, AC1
Pfeffer, G1
Majamaa, K2
Turnbull, DM1
Thorburn, D2
Chinnery, PF1
Stride, N1
Larsen, S1
Hey-Mogensen, M1
Sander, K1
Lund, JT1
Gustafsson, F1
Køber, L1
Dela, F1
Anglin, RE1
Rosebush, PI1
Noseworthy, MD1
Tarnopolsky, M1
Mazurek, MF1
Sarzi, E1
Angebault, C1
Seveno, M1
Gueguen, N1
Chaix, B1
Bielicki, G1
Boddaert, N1
Mausset-Bonnefont, AL1
Cazevieille, C1
Rigau, V1
Renou, JP1
Wang, J1
Delettre, C1
Brabet, P1
Puel, JL1
Hamel, CP1
Reynier, P1
Lenaers, G1
Pajares, S1
Arias, A1
García-Villoria, J1
Briones, P1
Ribes, A1
Kerr, DS1
Chinnery, P1
Turnbull, D1
Marmarou, A2
Signoretti, S2
Fatouros, P1
Aygok, GA2
Bullock, R1
Rodriguez, MC1
MacDonald, JR1
Mahoney, DJ1
Parise, G1
Fatouros, PP1
Portella, G1
Bullock, RM1
Bizzi, A1
Castelli, G1
Bugiani, M1
Barker, PB1
Herskovits, EH1
Danesi, U1
Erbetta, A1
Moroni, I1
Farina, L1
Uziel, G1

Clinical Trials (4)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
The Utility of pGz in Primary Mitochondrial Disorders[NCT05569122]Phase 190 participants (Anticipated)Interventional2023-03-22Recruiting
Development and Validation of a Myopathy Rating Scale in Mitochondrial Disease[NCT05250375]20 participants (Anticipated)Observational [Patient Registry]2017-03-24Recruiting
A Phase 2a Study of NAD+ Precursor Supplementation in Friedreich's Ataxia[NCT04817111]Phase 27 participants (Actual)Interventional2021-05-17Completed
A Phase II, Monocenter, Single Arm Study To Assess The Safety and Efficacy Of Combination Deoxycytidine and Deoxythymidine For Mitochondrial Depletion Disorders[NCT04802707]Phase 250 participants (Anticipated)Interventional2021-10-18Recruiting
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

Cardiac 31-Phosphorus-Magnetic Resonance Spectroscopy (MRS): Phosphocreatine (PCr)/Adenosine Tri-Phosphate (ATP) Ratio

Measure the within-participant change in PCr/ATP ratio before and after treatment with MIB-626. (NCT04817111)
Timeframe: Change from baseline to 14 days.

InterventionPCr to ATP ratio (Median)
Open Label - MIB-626-0.445

Concentration of Nicotinamide Adenine Dinucleotide (NAD+) in Whole Blood

Measure the concentration of NAD+ in whole blood before and after treatment with MIB-626. (NCT04817111)
Timeframe: Change from baseline to 14 days.

Interventionmicro-molar (Median)
Open Label - MIB-62649.9

Grip Strength

Assess within-participant changes in grip strength (via hand grip dynamometry) before and after treatment with MIB-626. (NCT04817111)
Timeframe: Change from baseline to 14 days.

Interventionkg (Median)
Open Label - MIB-626-0.65

Percentage of Individuals With Treatment-emergent Adverse Events as Assessed by Common Terminology Criteria for Adverse Events Version 5.0.

Safety will be monitored through collection of laboratory assessments (CBC, complete metabolic profile, lipid profile, HbA1c), vital signs (heart rate, blood pressure), and ECG, all of which will be reviewed for clinically relevant abnormalities, and standardized assessment of symptoms. We report the proportion of individuals who had at least one treatment-emergent adverse event of Grade 1 or higher. (NCT04817111)
Timeframe: 14 Days

InterventionParticipants (Count of Participants)
Open Label - MIB-6264

Reviews

9 reviews available for creatine and Electron Transport Chain Deficiencies, Mitochondrial

ArticleYear
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
Epilepsy, energy deficiency and new therapeutic approaches including diet.
    Pharmacology & therapeutics, 2014, Volume: 144, Issue:2

    Topics: Adenosine Triphosphatases; Adenosine Triphosphate; Astrocytes; Creatine; Diet, Ketogenic; Energy Int

2014
Clinical use of creatine in neuromuscular and neurometabolic disorders.
    Sub-cellular biochemistry, 2007, Volume: 46

    Topics: Animals; Body Composition; Charcot-Marie-Tooth Disease; Creatine; Disease Models, Animal; Glycogen S

2007
Creatine and its potential therapeutic value for targeting cellular energy impairment in neurodegenerative diseases.
    Neuromolecular medicine, 2008, Volume: 10, Issue:4

    Topics: Adenosine Triphosphate; Animals; Creatine; Dietary Supplements; Energy Metabolism; Humans; Mitochond

2008
Electron transfer mediators and other metabolites and cofactors in the treatment of mitochondrial dysfunction.
    Nutrition reviews, 2009, Volume: 67, Issue:8

    Topics: Acidosis, Lactic; Animals; Antioxidants; Carnitine; Creatine; Dietary Supplements; Electron Transpor

2009
Mitochondrial and metabolic-based protective strategies in Huntington's disease: the case of creatine and coenzyme Q.
    Reviews in the neurosciences, 2011, Dec-02, Volume: 23, Issue:1

    Topics: Animals; Creatine; Disease Models, Animal; Humans; Huntington Disease; Metabolic Diseases; Mitochond

2011
Treatment for mitochondrial disorders.
    The Cochrane database of systematic reviews, 2012, Apr-18, Issue:4

    Topics: Creatine; Dichloroacetic Acid; Humans; Mitochondrial Diseases; Randomized Controlled Trials as Topic

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
Treatment for mitochondrial disorders.
    The Cochrane database of systematic reviews, 2006, Jan-25, Issue:1

    Topics: Creatine; Dichloroacetic Acid; Humans; Mitochondrial Diseases; Sarcosine; Ubiquinone

2006

Trials

3 trials available for creatine and Electron Transport Chain Deficiencies, Mitochondrial

ArticleYear
Mitochondrial injury measured by proton magnetic resonance spectroscopy in severe head trauma patients.
    Acta neurochirurgica. Supplement, 2005, Volume: 95

    Topics: Adult; Aspartic Acid; Biomarkers; Brain; Brain Ischemia; Choline; Craniocerebral Trauma; Creatine; F

2005
Beneficial effects of creatine, CoQ10, and lipoic acid in mitochondrial disorders.
    Muscle & nerve, 2007, Volume: 35, Issue:2

    Topics: 8-Hydroxy-2'-Deoxyguanosine; Adolescent; Adult; Analysis of Variance; Antioxidants; Body Composition

2007
Assessment of mitochondrial impairment in traumatic brain injury using high-resolution proton magnetic resonance spectroscopy.
    Journal of neurosurgery, 2008, Volume: 108, Issue:1

    Topics: Adolescent; Adult; Aspartic Acid; Brain Injuries; Choline; Creatine; Female; Follow-Up Studies; Huma

2008

Other Studies

11 other studies available for creatine and Electron Transport Chain Deficiencies, Mitochondrial

ArticleYear
Plasma creatine as a marker of mitochondrial dysfunction.
    Medical hypotheses, 2018, Volume: 113

    Topics: Adenosine Triphosphate; Biomarkers; Creatine; Creatine Kinase, Mitochondrial Form; Electron Transpor

2018
Diagnostic value of MRS-quantified brain tissue lactate level in identifying children with mitochondrial disorders.
    European radiology, 2017, Volume: 27, Issue:3

    Topics: Adolescent; Biomarkers; Brain; Child; Child, Preschool; Creatine; Female; Humans; Infant; Infant, Ne

2017
Muscle oxidative phosphorylation quantitation using creatine chemical exchange saturation transfer (CrCEST) MRI in mitochondrial disorders.
    JCI insight, 2016, 11-03, Volume: 1, Issue:18

    Topics: Adult; Creatine; Exercise Test; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Mitoc

2016
Muscle oxidative phosphorylation quantitation using creatine chemical exchange saturation transfer (CrCEST) MRI in mitochondrial disorders.
    JCI insight, 2016, 11-03, Volume: 1, Issue:18

    Topics: Adult; Creatine; Exercise Test; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Mitoc

2016
Muscle oxidative phosphorylation quantitation using creatine chemical exchange saturation transfer (CrCEST) MRI in mitochondrial disorders.
    JCI insight, 2016, 11-03, Volume: 1, Issue:18

    Topics: Adult; Creatine; Exercise Test; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Mitoc

2016
Muscle oxidative phosphorylation quantitation using creatine chemical exchange saturation transfer (CrCEST) MRI in mitochondrial disorders.
    JCI insight, 2016, 11-03, Volume: 1, Issue:18

    Topics: Adult; Creatine; Exercise Test; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Mitoc

2016
Muscle oxidative phosphorylation quantitation using creatine chemical exchange saturation transfer (CrCEST) MRI in mitochondrial disorders.
    JCI insight, 2016, 11-03, Volume: 1, Issue:18

    Topics: Adult; Creatine; Exercise Test; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Mitoc

2016
Muscle oxidative phosphorylation quantitation using creatine chemical exchange saturation transfer (CrCEST) MRI in mitochondrial disorders.
    JCI insight, 2016, 11-03, Volume: 1, Issue:18

    Topics: Adult; Creatine; Exercise Test; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Mitoc

2016
Muscle oxidative phosphorylation quantitation using creatine chemical exchange saturation transfer (CrCEST) MRI in mitochondrial disorders.
    JCI insight, 2016, 11-03, Volume: 1, Issue:18

    Topics: Adult; Creatine; Exercise Test; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Mitoc

2016
Muscle oxidative phosphorylation quantitation using creatine chemical exchange saturation transfer (CrCEST) MRI in mitochondrial disorders.
    JCI insight, 2016, 11-03, Volume: 1, Issue:18

    Topics: Adult; Creatine; Exercise Test; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Mitoc

2016
Muscle oxidative phosphorylation quantitation using creatine chemical exchange saturation transfer (CrCEST) MRI in mitochondrial disorders.
    JCI insight, 2016, 11-03, Volume: 1, Issue:18

    Topics: Adult; Creatine; Exercise Test; Female; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Mitoc

2016
A plasma signature of human mitochondrial disease revealed through metabolic profiling of spent media from cultured muscle cells.
    Proceedings of the National Academy of Sciences of the United States of America, 2010, Jan-26, Volume: 107, Issue:4

    Topics: Adult; Animals; Biomarkers; Cell Line; Creatine; Culture Media; Electron Transport; Female; Humans;

2010
Therapeutic approaches to mitochondrial dysfunction in Parkinson's disease.
    Parkinsonism & related disorders, 2009, Volume: 15 Suppl 3

    Topics: Animals; Clinical Trials, Phase III as Topic; Creatine; Heat-Shock Proteins; Histocompatibility Anti

2009
X-linked creatine transporter deficiency presenting as a mitochondrial disorder.
    Journal of child neurology, 2010, Volume: 25, Issue:8

    Topics: Adolescent; Biomarkers; Creatine; Diagnosis, Differential; Genetic Diseases, X-Linked; Humans; Inclu

2010
Decreased mitochondrial oxidative phosphorylation capacity in the human heart with left ventricular systolic dysfunction.
    European journal of heart failure, 2013, Volume: 15, Issue:2

    Topics: Aged; Biopsy; Carnitine; Creatine; Creatine Kinase, Mitochondrial Form; Energy Metabolism; Fatty Aci

2013
Psychiatric symptoms correlate with metabolic indices in the hippocampus and cingulate in patients with mitochondrial disorders.
    Translational psychiatry, 2012, Nov-13, Volume: 2

    Topics: Adult; Aged; Anxiety; Aspartic Acid; Case-Control Studies; Caudate Nucleus; Creatine; Female; Glutam

2012
The human OPA1delTTAG mutation induces premature age-related systemic neurodegeneration in mouse.
    Brain : a journal of neurology, 2012, Volume: 135, Issue:Pt 12

    Topics: Acoustic Stimulation; Age Factors; Aging, Premature; Animals; Aspartic Acid; Chi-Square Distribution

2012
Role of creatine as biomarker of mitochondrial diseases.
    Molecular genetics and metabolism, 2013, Volume: 108, Issue:2

    Topics: Adolescent; Adult; Biomarkers; Child; Child, Preschool; Creatine; Humans; Infant; Infant, Newborn; L

2013
Classification of childhood white matter disorders using proton MR spectroscopic imaging.
    AJNR. American journal of neuroradiology, 2008, Volume: 29, Issue:7

    Topics: Adrenoleukodystrophy; Adult; Alexander Disease; Aspartic Acid; Brain; Child; Child, Preschool; Choli

2008