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Beneficial effects of creatine, CoQ 10 , and lipoic acid in mitochondrial disorders
Author(s) -
Rodriguez M. Christine,
MacDonald Jay R.,
Mahoney Douglas J.,
Parise Gianni,
Beal M. Flint,
Tarnopolsky Mark A.
Publication year - 2007
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.20688
Subject(s) - lactic acidosis , mitochondrial myopathy , creatine monohydrate , melas syndrome , mitochondrial encephalomyopathy , creatine , chronic progressive external ophthalmoplegia , medicine , kearns–sayre syndrome , mitochondrial disease , mitochondrial dna , coenzyme q – cytochrome c reductase , endocrinology , crossover study , heteroplasmy , mitochondrion , anaerobic exercise , lipoic acid , gastroenterology , placebo , biology , biochemistry , pathology , physical therapy , alternative medicine , gene , cytochrome c , antioxidant
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