The DOI matches its Crossref record; 39 of the 39 references it cites were found in a registry or an index.
Beneficial effects of creatine, CoQ10, and lipoic acid in mitochondrial disorders
Abstract
Mitochondrial disorders share common cellular consequences: (1) decreased ATP production; (2) increased reliance on alternative anaerobic energy sources; and (3) increased production of reactive oxygen species. The purpose of the present study was to determine the effect of a combination therapy (creatine monohydrate, coenzyme Q 10, and lipoic acid to target the above‐mentioned cellular consequences) on several outcome variables using a randomized, double‐blind, placebo‐controlled, crossover study design in patients with mitochondrial cytopathies. Three patients had mitochondrial encephalopathy, lactic acidosis, and stroke‐like episodes (MELAS), four had mitochondrial DNA deletions (three patients with chronic progressive external ophthalmoplegia and one with Kearns–Sayre syndrome), and nine had a variety of other mitochondrial diseases not falling into the two former groups. The combination therapy resulted in lower resting plasma lactate and urinary 8‐isoprostanes, as well as attenuation of the decline in peak ankle dorsiflexion strength in all patient groups, whereas higher fat‐free mass was observed only in the MELAS group. Together, these results suggest that combination therapies targeting multiple final common pathways of mitochondrial dysfunction favorably influence surrogate markers of cellular energy dysfunction. Future studies with larger sample sizes in relatively homogeneous groups will be required to determine whether such combination therapies influence function and quality of life. Muscle Nerve, 2006
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“We found that α-lipoic acid exerts significant neuroprotective effects in transgenic mouse models of HD and ALS128, 187, 188.”
From Mitochondrial Approaches for Neuroprotection (2008) · cites this paper as [128]
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Metadata
- Authors
- Rodriguez, M. C., MacDonald, J. R., Mahoney, D. J., Parise, G., Beal, M. F., & Tarnopolsky, M. A.
- Affiliations
- McMaster University (CA)
- Cornell University (US)
- Corresponding author
- Tarnopolsky, Mark A.
- Venue
- Muscle & Nerve
- Publisher
- Wiley
- Published
- 2007-01-18
- Volume
- 35
- Issue
- 2
- Pages
- 235-242
- ISSN
- 0148-639X (ISSN-L)
- 1097-4598
- OpenAlex
- W2063874785
- Cited by
- 255
- References
- 39
- Citations by year
- 2026: 4 · 2025: 14 · 2024: 4 · 2023: 8 · 2022: 11 · 2021: 12 · 2020: 17 · 2019: 17 · 2018: 14 · 2017: 14 · 2016: 11 · 2015: 16 · 2014: 17 · 2013: 14 · 2012: 13
- FWCI
- 4.90
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Muscle & Nerve (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Coenzyme Q10 studies and effects
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Keywords
- mitochondrial disease, creatine monohydrate, coenzyme Q10, alpha-lipoic acid, combination therapy, MELAS, mitochondrial DNA deletions, chronic progressive external ophthalmoplegia, Kearns-Sayre syndrome, plasma lactate, reactive oxygen species, anaerobic metabolism, fat-free mass, ankle dorsiflexion strength, ATP production
- MeSH terms
- 8-Hydroxy-2'-Deoxyguanosine, Adolescent, Adult, Analysis of Variance, Antioxidants, Body Composition, Child, Coenzymes, Creatine, Creatinine, Deoxyguanosine, Double-Blind Method, Female, Humans, Thioctic Acid, Male, Middle Aged, Ubiquinone, Dinoprost, Cross-Over Studies, Mitochondrial Diseases
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