Beneficial effects of creatine, CoQ10, and lipoic acid in mitochondrial disorders

Elevation of creatine in resting and exercised muscle of normal subjects by creatine supplementation

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Isoprostanes: markers and mediators of oxidative stress

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Lipoic Acid as a Potential Therapy for Chronic Diseases Associated with Oxidative Stress

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Direct antioxidant properties of creatine

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F 2 -Isoprostanes as markers of oxidative stress in vivo : An overview

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Creatine Supplementation Enhances Isometric Strength and Body Composition Improvements Following Strength Exercise Training in Older Adults

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A randomized, controlled trial of creatine monohydrate in patients with mitochondrial cytopathies

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Potential for creatine and other therapies targeting cellular energy dysfunction in neurological disorders

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Isoprostanes and related products of lipid peroxidation in neurodegenerative diseases

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Creatine supplementation affords cytoprotection in oxidatively injured cultured mammalian cells via direct antioxidant activity

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Mitochondrial DNA sequence associations with dementia and amyloid-β in elderly African Americans

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Cardiovascular Manifestations of Mitochondrial Disease

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Treatment of Mitochondrial Disorders

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Creatine for treating muscle disorders

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Mitochondrial DNA replication and repair defects: Clinical phenotypes and therapeutic interventions

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Targeting autophagy and mitophagy for mitochondrial diseases treatment

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Symptoms of somatization as a rapid screening tool for mitochondrial dysfunction in depression

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Genetics of ischaemic stroke

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Effects of 12 Weeks of Essential Amino Acids (EAA)-Based Multi-Ingredient Nutritional Supplementation on Muscle Mass, Muscle Strength, Muscle Power and Fatigue in Healthy Elderly Subjects: A Randomized Controlled Double-Blind Study

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How Can We Treat Mitochondrial Encephalomyopathies? Approaches to Therapy

Horváth, R., Gorman, G. S., & Chinnery, P. F. (2008) · Neurotherapeutics · doi:10.1016/j.nurt.2008.07.002 · Cited by 36

Mitochondrial disorders and drugs: what every physician should know

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Therapeutic Strategies for Mitochondrial Disorders

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Role of Creatine Supplementation on Exercise‐Induced Cardiovascular Function and Oxidative Stress

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The investigation and management of metabolic myopathies

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Update on Pharmacological Treatment of Progressive Myoclonus Epilepsies

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Clinical syndromes associated with mtDNA mutations: where we stand after 30 years

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Novel Multi-Ingredient Supplement Facilitates Weight Loss and Improves Body Composition in Overweight and Obese Individuals: A Randomized, Double-Blind, Placebo-Controlled Clinical Trial

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Nutritional Interventions for Mitochondrial OXPHOS Deficiencies: Mechanisms and Model Systems

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Introduction – Creatine: Cheap Ergogenic Supplement with Great Potential for Health and Disease

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Treatment of Leber hereditary optic neuropathy

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Delivery Systems for Mitochondrial Gene Therapy: A Review

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Mitochondria and oxidative stress in epilepsy: advances in antioxidant therapy

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Administration of CoQ10 analogue ameliorates dysfunction of the mitochondrial respiratory chain in a mouse model of Angelman syndrome

Llewellyn, K. J., Nalbandian, A., Gómez, A. E., Don, W., Walker, N., & Kimonis, V. E. (2015) · Neurobiology of Disease · doi:10.1016/j.nbd.2015.01.005 · Cited by 31

Drugs and mitochondrial diseases: 40 queries and answers

Mancuso, M., Orsucci, D., Filosto, M., Simoncini, C., & Siciliano, G. (2012) · Expert Opinion on Pharmacotherapy · doi:10.1517/14656566.2012.657177 · Cited by 31

Mitochondrial disease patients' perception of dietary supplements' use

Karaa, A., Kriger, J., Grier, J., Holbert, A., Thompson, J. L. P., Parikh, S., & Hirano, M. (2016) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2016.07.005 · Cited by 30

Current Experience in Testing Mitochondrial Nutrients in Disorders Featuring Oxidative Stress and Mitochondrial Dysfunction: Rational Design of Chemoprevention Trials

Pagano, G., Talamanca, A. A., Castello, G., Cordero, M. D., d’Ischia, M., Gadaleta, M. N., Pallardó, F. V., Petrović, S. Ž., Tiano, L., & Zatterale, A. (2014) · International Journal of Molecular Sciences · doi:10.3390/ijms151120169 · Cited by 26

Appendicular skeletal muscle mass: A more sensitive biomarker of disease severity than BMI in adults with mitochondrial diseases

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Aging-Related Disorders and Mitochondrial Dysfunction: A Critical Review for Prospect Mitoprotective Strategies Based on Mitochondrial Nutrient Mixtures

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Therapy Prospects for Mitochondrial DNA Maintenance Disorders

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Treatment of central nervous system manifestations in mitochondrial disorders

Finsterer, J. (2010) · European Journal of Neurology · doi:10.1111/j.1468-1331.2010.03086.x · Cited by 26

Systemic administration of AAV-Slc25a46 mitigates mitochondrial neuropathy in Slc25a46−/− mice

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Defects in Metabolism of Carbohydrates

Kishnani, P. S., & Chen, Y. (2011) · Elsevier eBooks · doi:10.1016/b978-1-4377-0755-7.00081-6 · Cited by 26

Creatine monohydrate supplementation for older adults and clinical populations

Candow, D. G., Ostojić, S. M., Chilibeck, P. D., Longobardi, I., Gualano, B., Tarnopolsky, M. A., Wallimann, T., Moriarty, T. A., Kreider, R. B., Forbes, S. C., et al. (2025) · Journal of the International Society of Sports Nutrition · doi:10.1080/15502783.2025.2534130 · Cited by 37

The Protective Effects of Alpha-Lipoic Acid and Coenzyme Q10Combination on Ovarian Ischemia-Reperfusion Injury: An Experimental Study

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Oxidative stress biomarkers in urine and plasma of rabbits with partial bladder outlet obstruction

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Design and implementation of the first randomized controlled trial of coenzyme Q10 in children with primary mitochondrial diseases

Stacpoole, P. W., deGrauw, T. J., Feigenbaum, A. S. J., Hoppel, C. L., Kerr, D. S., McCandless, S. E., Miles, M. V., Robinson, B. H., & Tang, P. (2012) · Mitochondrion · doi:10.1016/j.mito.2012.09.005 · Cited by 25

Systemic effect of dietary lipid levels and α-lipoic acid supplementation on nutritional metabolism in zebrafish (Danio rerio): focusing on the transcriptional level

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Why are there no proven therapies for genetic mitochondrial diseases?

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Neuropsychiatric Features in Primary Mitochondrial Disease

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Electron transfer mediators and other metabolites and cofactors in the treatment of mitochondrial dysfunction

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Mitochondrial Function Is Related to Alterations at Brain SPECT in Depressed Patients

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Mitochondria: Prospective Targets for Neuroprotection in Parkinson’s Disease

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Gender-specific association of oxidative stress and immune response in septic shock mortality using NMR-based metabolomics

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Metabolic Myopathies

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Mitochondria as promising targets for nutritional interventions aiming to improve performance and longevity of sows

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Cellular Stress in the Pathogenesis of Muscular Disorders—From Cause to Consequence

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Mitochondrial disorders and the eye

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How do you treat patients with myalgia who take statins?

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Metabolic Myopathies: Clinical Features and Diagnostic Approach

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A case of hypopituitarism accompanying Kearns-Sayre syndrome treated with human chorionic gonadotropin: A case report and literature review

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Complex III deficiency due to an in-frame MT-CYB deletion presenting as ketotic hypoglycemia and lactic acidosis

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Beneficial Effects of a Q-ter® Based Nutritional Mixture on Functional Performance, Mitochondrial Function, and Oxidative Stress in Rats

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Effect of rapamycin on mitochondria and lysosomes in fibroblasts from patients with mtDNA mutations

Cheema, N., Cameron, J. M., & Hood, D. A. (2021) · American Journal of Physiology-Cell Physiology · doi:10.1152/ajpcell.00471.2020 · Cited by 18

The Acute Effect of Multi-Ingredient Antioxidant Supplementation following Ionizing Radiation

Xhuti, D., Rebalka, I. A., Minhas, M., May, L. L., Murphy, K. J., Nederveen, J. P., & Tarnopolsky, M. A. (2023) · Nutrients · doi:10.3390/nu15010207 · Cited by 17

Recent advances in small molecules for improving mitochondrial disorders

Meng, L., & Wu, G. (2023) · RSC Advances · doi:10.1039/d3ra03313a · Cited by 18

Mitochondrial Tolerance to Drugs and Toxic Agents in Ageing and Disease

Suski, J. M., Lebiedzińska, M., Machado, N. G., Oliveira, P. J., Pinton, P., Duszyński, J., & Wieckowski, M. R. (2011) · Current Drug Targets · doi:10.2174/138945011795529029 · Cited by 16

Heteroplasmy Shifting as Therapy for Mitochondrial Disorders

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Friedreich Ataxia: current state-of-the-art, and future prospects for mitochondrial-focused therapies

Pallardó, F. V., Pagano, G., Rodríguez, L. R., González‐Cabo, P., Lyakhovich, A., & Trifuoggi, M. (2020) · Translational research · doi:10.1016/j.trsl.2020.08.009 · Cited by 16

Therapeutical Management and Drug Safety in Mitochondrial Diseases—Update 2020

Gruosso, F., Montano, V., Simoncini, C., Siciliano, G., & Mancuso, M. (2020) · Journal of Clinical Medicine · doi:10.3390/jcm10010094 · Cited by 16

Targeted Biomedical Treatment for Autism Spectrum Disorders

Pacheva, I. H., & Ivanov, I. S. (2019) · Current Pharmaceutical Design · doi:10.2174/1381612825666191205091312 · Cited by 17

FSHD Therapeutic Strategies: What Will It Take to Get to Clinic?

Himeda, C. L., & Jones, P. L. (2022) · Journal of Personalized Medicine · doi:10.3390/jpm12060865 · Cited by 18

The Elusive Magic Pill: Finding Effective Therapies for Mitochondrial Disorders

Goldstein, A. C., & Wolfe, L. A. (2013) · Neurotherapeutics · doi:10.1007/s13311-012-0175-0 · Cited by 16

Mitochondrial Disorders of the Nervous System: A Review

Set, K. K., Sen, K., Huq, A. H. M. M., & Agarwal, R. L. (2019) · Clinical Pediatrics · doi:10.1177/0009922818821890 · Cited by 14

Mitigating the pro-oxidant state and melanogenesis of Retinitis pigmentosa: by counteracting mitochondrial dysfunction

Pagano, G., Pallardó, F. V., Lyakhovich, A., Tiano, L., & Trifuoggi, M. (2021) · Cellular and Molecular Life Sciences · doi:10.1007/s00018-021-04007-1 · Cited by 15

Effect of Co-enzyme Q10 and Alpha-Lipoic Acid on Response of Rabbit Urinary Bladder to Repetitive Stimulation and In Vitro Ischemia

Lin, W., Rehfuss, A. W., Schuler, C., & Levin, R. M. (2008) · Urology · doi:10.1016/j.urology.2007.11.074 · Cited by 15

Kearns-Sayre syndrome presenting as isolated growth failure

Holloman, C., Wolfe, L. A., Gahl, W. A., & Boerkoel, C. F. (2013) · BMJ Case Reports · doi:10.1136/bcr-2012-007272 · Cited by 15

Keep the fire burning: Current avenues in the quest of treating mitochondrial disorders

Tischner, C., & Wenz, T. (2015) · Mitochondrion · doi:10.1016/j.mito.2015.06.002 · Cited by 14

Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Episodes (MELAS)

Goldstein, A. C., & Servidei, S. (2019) · Springer eBooks · doi:10.1007/978-3-030-05517-2_5 · Cited by 14

Expanding the Phenotype: Neurodevelopmental Disorder, Mitochondrial, With Abnormal Movements and Lactic Acidosis, With or Without Seizures (NEMMLAS) due to WARS2 Biallelic Variants, Encoding Mitochondrial Tryptophanyl-tRNA Synthase

Virdee, M., Swarnalingam, E., Kozenko, M., Tarnopolsky, M. A., & Jones, K. (2019) · Journal of Child Neurology · doi:10.1177/0883073819854604 · Cited by 13

Mitochondrial cardiomyopathies: pathogenesis, diagnosis, and treatment

Maack, C., Dudek, J., Bertero, E., Tampakakis, E., & Vernon, H. J. (2025) · European Heart Journal · doi:10.1093/eurheartj/ehaf491 · Cited by 12

The Role of Primary Mitochondrial Disorders in Hearing Impairment: An Overview

Fancello, V., Fancello, G., Palma, S., Monzani, D., Genovese, E., Bianchini, C., & Ciorba, A. (2023) · Medicina · doi:10.3390/medicina59030608 · Cited by 13

Assessing Bioenergetic Compromise in Autism Spectrum Disorder With 31 P Magnetic Resonance Spectroscopy

Golomb, B. A., Erickson, L. C., Zeeland, A. A. S., Koperski, S., Haas, R., Wallace, D. C., Naviaux, R. K., Lincoln, A. J., Reiner, G., & Hamilton, G. (2013) · Journal of Child Neurology · doi:10.1177/0883073813498466 · Cited by 12

Rapamycin rescues mitochondrial dysfunction in cells carrying the m.8344A > G mutation in the mitochondrial tRNALys

Capristo, M., Dotto, V. D., Tropeano, C. V., Fiorini, C., Caporali, L., Morgia, C. L., Valentino, M. L., Montopoli, M., Carelli, V., & Maresca, A. (2022) · Molecular Medicine · doi:10.1186/s10020-022-00519-z · Cited by 11

Genetic Stroke Syndromes

Barrett, K. M., & Meschia, J. F. (2014) · CONTINUUM Lifelong Learning in Neurology · doi:10.1212/01.con.0000446109.20539.68 · Cited by 11

Influence of metformin on mitochondrial subproteome in the brain of apoE knockout mice

Suski, M., Olszanecki, R., Chmura, Ł., Stachowicz, A., Madej, J., Okoń, K., Adamek, D., & Korbut, R. (2015) · European Journal of Pharmacology · doi:10.1016/j.ejphar.2015.12.036 · Cited by 10

Unusual exocrine complication of pancreatitis in mitochondrial disease

Ishiyama, A., Komaki, H., SAITO, T. K., Saito, Y., Nakagawa, E., Sugai, K., Itagaki, Y., Matsuzaki, K., Nakura, M., Nishino, I., et al. (2012) · Brain and Development · doi:10.1016/j.braindev.2012.10.015 · Cited by 10

Autophagy and Mitochondrial Encephalomyopathies

Zhang, X., Zheng, Y., & Chen, Z. (2020) · Advances in experimental medicine and biology · doi:10.1007/978-981-15-4272-5_6 · Cited by 10

Multiple Neurologic, Psychiatric, and Endocrine Complaints in a Young Woman: A Case Discussion and Review of the Clinical Features and Management of Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Stroke

Bhuvaneswar, C. G., Goetz, J. L., & Stern, T. A. (2008) · The Primary Care Companion to The Journal of Clinical Psychiatry · doi:10.4088/pcc.v10n0309 · Cited by 10

PARS2-associated mitochondrial disease: A case report of a patient with prolonged survival and literature review

Almuqbil, M. A., Vernon, H. J., Ferguson, M., & Kline, A. D. (2020) · Molecular Genetics and Metabolism Reports · doi:10.1016/j.ymgmr.2020.100613 · Cited by 9

Clinical Therapeutic Management of Human Mitochondrial Disorders

Finsterer, J. (2020) · Pediatric Neurology · doi:10.1016/j.pediatrneurol.2020.07.004 · Cited by 9

Oral Feeding of an Antioxidant Cocktail as a Therapeutic Strategy in a Mouse Model of Rett Syndrome: Merits and Limitations of Long-Term Treatment

Baroncelli, L., Stefanie, A., Rinne, L., Schuster, A.-K., Brand, V., Kempkes, B., Dietrich, K., & Müller, M. (2022) · Antioxidants · doi:10.3390/antiox11071406 · Cited by 8

The current landscape for the treatment of mitochondrial disorders

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New avenues for therapy in mitochondrial optic neuropathies

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Retinal Gatekeepers: Molecular Mechanism and Therapeutic Role of Cysteine and Selenocysteine

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Mitochondrial Myopathy, Encephalopathy, Lactic Acidosis, and Stroke-Like Episodes (MELAS)

Marin, S. E., & Haas, R. (2015) · Elsevier eBooks · doi:10.1016/b978-0-12-800877-5.00002-4 · Cited by 7

Metabolic Cardiomyopathy and Mitochondrial Disorders in the Pediatric Intensive Care Unit

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