Cited by 255Mitochondrial Function and PathologyCoenzyme Q10 studies and effectsMetabolism and Genetic Disorders

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

Rodriguez, M. Christine, MacDonald, Jay R., Mahoney, Douglas J., Parise, Gianni, Beal, M. Flint, Tarnopolsky, Mark A.·2007·Muscle & Nerve

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

Integrity signals

Registry

Crossref · 10.1002/mus.20688

Cites

39 of 39 references verified0 citation errors · 0 not checkable

Statistics

Recomputing p-values needs the paper’s full text.

Claims the paper makes

This paper’s full text isn’t openly available, so its claims and self-check weren’t run. The registry and citation checks still apply.

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?

  • α-lipoic acid exerts significant neuroprotective effects in transgenic mouse models of HD and ALS.

    “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]

Cites

Flagged first, then by citations

Cited by

Sorted by citations

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