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
Overview
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
In the citing paper
“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]
Citations
Cites
Elevation of creatine in resting and exercised muscle of normal subjects by creatine supplementation
Harris, R. C., Söderlund, K., & Hultman, E. (1992) · Clinical Science · doi:10.1042/cs0830367 · Cited by 1,168
Isoprostanes: markers and mediators of oxidative stress
Montuschi, P., Barnes, P. J., & Roberts, L. J. (2004) · The FASEB Journal · doi:10.1096/fj.04-2330rev · Cited by 747
Lipoic Acid as a Potential Therapy for Chronic Diseases Associated with Oxidative Stress
Smith, A. R. W., Shenvi, S. V., Widlansky, M. E., Suh, J.-I., & Hagen, T. M. (2004) · Current Medicinal Chemistry · doi:10.2174/0929867043365387 · Cited by 461
Direct antioxidant properties of creatine
Lawler, J. M., Barnes, W. S., Wu, G., Song, W., & Demaree, S. (2002) · Biochemical and Biophysical Research Communications · doi:10.1006/bbrc.2001.6164 · Cited by 448
F 2 -Isoprostanes as markers of oxidative stress in vivo : An overview
Milne, G. L., Musiek, E. S., & Morrow, J. D. (2005) · Biomarkers · doi:10.1080/13547500500216546 · Cited by 323
Brose, A., Parise, G., & Tarnopolsky, M. A. (2003) · The Journals of Gerontology Series A: Biological Sciences and Medical Sciences · doi:10.1093/gerona/58.1.b11 · Cited by 274
Self-citationA randomized, controlled trial of creatine monohydrate in patients with mitochondrial cytopathies
Tarnopolsky, M. A., Roy, B. D., & MacDonald, J. R. (1997) · Muscle & Nerve · doi:10.1002/(sici)1097-4598(199712)20:12 · Cited by 246
Self-citationTarnopolsky, M. A., & Beal, M. F. (2001) · Annals of Neurology · doi:10.1002/ana.1028 · Cited by 244
Self-citationIsoprostanes and related products of lipid peroxidation in neurodegenerative diseases
Montine, K. S., Quinn, J. F., Zhang, J., Fessel, J. P., Roberts, L. J., Morrow, J. D., & Montine, T. J. (2003) · Chemistry and Physics of Lipids · doi:10.1016/j.chemphyslip.2003.10.010 · Cited by 242
Sestili, P., Martinelli, C., Bravi, G., Piccoli, G., Curci, R., Battistelli, M., Falcieri, E., Agostini, D., Gioacchini, A. M., & Stocchi, V. (2006) · Free Radical Biology and Medicine · doi:10.1016/j.freeradbiomed.2005.10.035 · Cited by 220
Flagged first, then by citations
Cited by
Sorted by citations
Gorman, G. S., Chinnery, P. F., DiMauro, S., Hirano, M., Koga, Y., McFarland, R., Suomalainen, A., Thorburn, D. R., Zeviani, M., & Turnbull, D. M. (2016) · Nature Reviews Disease Primers · doi:10.1038/nrdp.2016.80 · Cited by 1,650
The creatine kinase system and pleiotropic effects of creatine
Wallimann, T., Tokarska-Schlattner, M., & Schlattner, U. (2011) · Amino Acids · doi:10.1007/s00726-011-0877-3 · Cited by 797
Mitochondrial optic neuropathies—Disease mechanisms and therapeutic strategies
Yu-Wai-Man, P., Griffiths, P. G., & Chinnery, P. F. (2011) · Progress in Retinal and Eye Research · doi:10.1016/j.preteyeres.2010.11.002 · Cited by 633
Parikh, S., Goldstein, A., Koenig, M. K., Scaglia, F., Enns, G. M., Saneto, R., Anselm, I., Cohen, B. H., Falk, M. J., Greene, C., et al. (2015) · Genetics in Medicine · doi:10.1038/gim.2014.177 · Cited by 607
Self-citationMELAS syndrome: Clinical manifestations, pathogenesis, and treatment options
El‐Hattab, A. W., Adesina, A. M., Jones, J. Y., & Scaglia, F. (2015) · Molecular Genetics and Metabolism · doi:10.1016/j.ymgme.2015.06.004 · Cited by 635
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
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
