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Condition

Mitochondrial Myopathy

Mitochondrial myopathy is a muscle condition where mitochondria, the cell's energy makers, work poorly, so muscles tire easily. Creatine and L-carnitine have the most research, and small studies found symptoms eased with creatine and exercise capacity improved with L-carnitine. The evidence is limited, though: the creatine study followed only four children, and the carnitine work twelve people.

Sources: PMID 10454733

Supplements studied
2
Graded outcomes
2
Trials
2
People studied
380

Evidence by supplement

  • Strong
  • Moderate
  • Limited
  • Very limited

2 supplements

Creatine

Creatine: outcomes studied for Mitochondrial Myopathy
GradeOutcomeEffectSizeStudiesPeopleStudies list
Mitochondrial Cytopathies Symptoms

Small Improvement

Improves (the measure goes down)Small effect1 study4 people

Studies that measured mitochondrial cytopathies symptoms

Power OutputImproves (the measure goes up)44 studies277 people

Studies that measured power output

and 41 more on the Creatine page

Muscular Dystrophy SymptomsImproves (the measure goes down)5 studies183 people

Studies that measured muscular dystrophy symptoms

and 2 more on the Creatine page

Subjective Well-BeingImproves (the measure goes up)3 studies4 people

Studies that measured subjective well-being

Neuromuscular FunctionImproves (the measure goes up)120 people

L-Carnitine

L-Carnitine: outcomes studied for Mitochondrial Myopathy
GradeOutcomeEffectSizeStudiesPeopleStudies list
Exercise Capacity

Small Improvement

Improves (the measure goes up)Small effect12 people
Muscle MassImproves (the measure goes up)3 studies12 people

Studies that measured muscle mass

Respiratory Exchange RatioImproves (the measure goes up)2 studies12 people

Studies that measured respiratory exchange ratio

Oxygen Uptake

Improvement

Improves (the measure goes up)1 study

Studies that measured oxygen uptake

Heart RateImproves (the measure goes down)12 people
Lung FunctionImproves (the measure goes up)12 people
StrengthImproves (the measure goes up)12 people

Key findings

  • Mitochondrial Cytopathies SymptomsCreatineImproves (the measure goes down)
  • Exercise CapacityL-CarnitineImproves (the measure goes up)

The Mitochondrial Myopathy protocol

2 primary supplements · 3 supporting supplements

See the full protocol

Studies cited

58 studies from PubMed

  1. Creatine supplementation improves muscle strength in patients with congestive heart failurePharmazie · PMID 16599263
  2. Comparison of creatine monohydrate and carbohydrate supplementation on repeated jump height performanceJ Strength Cond Res · PMID 18545204
  3. Effect of Creatine Supplementation on Jumping Performance in Elite Volleyball PlayersInt J Sports Physiol Perform · PMID 21941005
  4. Supplementation with creatine monohydrate in children with mitochondrial encephalomyopathiesMuscle Nerve · PMID 10454733
  5. Creatine monohydrate in DM2/PROMM: a double-blind placebo-controlled clinical study. Proximal myotonic myopathyNeurology · PMID 12578937
  6. Oral creatine supplementation facilitates the rehabilitation of disuse atrophy and alters the expression of muscle myogenic factors in humansJ Physiol · PMID 11600695
  7. Creatine monohydrate in myotonic dystrophy: a double-blind, placebo-controlled clinical studyJ Neurol · PMID 12529796
  8. Creatine monohydrate supplementation does not increase muscle strength, lean body mass, or muscle phosphocreatine in patients with myotonic dystrophy type 1Muscle Nerve · PMID 14694498
  9. Resistance training with creatine monohydrate improves upper-body strength in patients with Parkinson disease: a randomized trialNeurorehabil Neural Repair · PMID 17312085
  10. Effects of acute creatine loading with or without carbohydrate on repeated bouts of maximal swimming in high-performance swimmersJ Strength Cond Res · PMID 15903360
  11. Creatine supplementation improves the anaerobic performance of elite junior fin swimmersActa Physiol Hung · PMID 19706374
  12. Low dose creatine supplementation enhances sprint phase of 400 meters swimming performanceJ Med Assoc Thai · PMID 16083193
  13. Effect of creatine on swimming velocity, body composition and hydrodynamic variablesJ Sports Med Phys Fitness · PMID 17369799
  14. Effect of oral creatine supplementation on single-effort sprint performance in elite swimmersInt J Sport Nutr · PMID 8876342
  15. Creatine supplementation, sleep deprivation, cortisol, melatonin and behaviorPhysiol Behav · PMID 17046034
  16. Effect of creatine supplementation and sleep deprivation, with mild exercise, on cognitive and psychomotor performance, mood state, and plasma concentrations of catecholamines and cortisolPsychopharmacology · PMID 16416332
  17. Physiological responses to short-term exercise in the heat after creatine loadingMed Sci Sports Exerc · PMID 11445756
  18. Mg2+-creatine chelate and a low-dose creatine supplementation regimen improve exercise performanceJ Strength Cond Res · PMID 15142029
  19. Effects of creatine supplementation and three days of resistance training on muscle strength, power output, and neuromuscular functionJ Strength Cond Res · PMID 17685691
  20. Effect of caffeine ingestion after creatine supplementation on intermittent high-intensity sprint performanceEur J Appl Physiol · PMID 21207054
  21. The effects of four weeks of creatine supplementation and high-intensity interval training on cardiorespiratory fitness: a randomized controlled trialJ Int Soc Sports Nutr · PMID 19909536
  22. Effects of high dose oral creatine supplementation on anaerobic capacity of elite wrestlersJ Sports Med Phys Fitness · PMID 14767410
  23. Effects of two and five days of creatine loading on muscular strength and anaerobic power in trained athletesJ Strength Cond Res · PMID 19387386
  24. Effects of oral creatine and resistance training on serum myostatin and GASP-1Mol Cell Endocrinol · PMID 20026378
  25. Effects of creatine monohydrate supplementation on body composition and strength indices in experienced resistance trained womenJ Strength Cond Res · PMID 17194243
  26. Creatine as a metabolic controller of skeletal muscles structure and function in strength exercises in humansRoss Fiziol Zh Im I M Sechenova · PMID 16613062
  27. The effects of creatine supplementation on muscular performance and body composition responses to short-term resistance training overreachingEur J Appl Physiol · PMID 14685870
  28. Effects of creatine monohydrate and polyethylene glycosylated creatine supplementation on muscular strength, endurance, and power outputJ Strength Cond Res · PMID 19387397
  29. Effect of different frequencies of creatine supplementation on muscle size and strength in young adultsJ Strength Cond Res · PMID 21512399
  30. The effects of pre versus post workout supplementation of creatine monohydrate on body composition and strengthJ Int Soc Sports Nutr · PMID 23919405
  31. The effects of supplementation with creatine and protein on muscle strength following a traditional resistance training program in middle-aged and older menJ Nutr Health Aging · PMID 20126965
  32. Effect of thirty days of creatine supplementation with phosphate salts on anaerobic working capacity and body weight in menJ Strength Cond Res · PMID 18438234
  33. The effects of polyethylene glycosylated creatine supplementation on muscular strength and powerJ Strength Cond Res · PMID 21068676
  34. Effect of short-term creatine supplementation on neuromuscular functionMed Sci Sports Exerc · PMID 19727018
  35. Effect of creatine and beta-alanine supplementation on performance and endocrine responses in strength/power athletesInt J Sport Nutr Exerc Metab · PMID 17136944
  36. Effects of four weeks of high-intensity interval training and creatine supplementation on critical power and anaerobic working capacity in college-aged menJ Strength Cond Res · PMID 19675499
  37. Does nutritional supplementation influence adaptability of muscle to resistance training in men aged 48 to 72 yearsJ Geriatr Phys Ther · PMID 16236227
  38. Effect of creatine ingestion after exercise on muscle thickness in males and femalesMed Sci Sports Exerc · PMID 15595301
  39. Creatine monohydrate increases strength in patients with neuromuscular diseaseNeurology · PMID 10078740
  40. Creatine supplementation and physical training in patients with COPD: a double blind, placebo-controlled studyInt J Chron Obstruct Pulmon Dis · PMID 18044100
  41. The Paradoxical Effect of Creatine Monohydrate on Muscle Damage Markers: A Systematic Review and Meta-AnalysisSports Med · PMID 35218552
  42. Effects of creatine on body composition and strength gains after 4 weeks of resistance training in previously nonresistance-trained humansInt J Sport Nutr Exerc Metab · PMID 14967873
  43. The effects of creatine monohydrate supplementation with and without D-pinitol on resistance training adaptationsJ Strength Cond Res · PMID 19858753
  44. Creatine supplementation improves muscular performance in older womenEur J Appl Physiol · PMID 17943308
  45. Effects of creatine supplementation in taekwondo practitionersNutr Hosp · PMID 23822690
  46. Effect of creatine and weight training on muscle creatine and performance in vegetariansMed Sci Sports Exerc · PMID 14600563
  47. Creatine fails to augment the benefits from resistance training in patients with HIV infection: a randomized, double-blind, placebo-controlled studyPLoS One · PMID 19242554
  48. Effect of in-season creatine supplementation on body composition and performance in rugby union football playersAppl Physiol Nutr Metab · PMID 18059577
  49. The effects of creatine ethyl ester supplementation combined with heavy resistance training on body composition, muscle performance, and serum and muscle creatine levelsJ Int Soc Sports Nutr · PMID 19228401
  50. Creatine, arginine alpha-ketoglutarate, amino acids, and medium-chain triglycerides and endurance and performanceInt J Sport Nutr Exerc Metab · PMID 19033611
  51. Comparison of creatine ingestion and resistance training on energy expenditure and limb blood flowMetabolism · PMID 11735088
  52. Creatine monohydrate enhances strength and body composition in Duchenne muscular dystrophyNeurology · PMID 15159476
  53. Creatine for treating muscle disorders.Cochrane Database Syst Rev · PMID 23740606
  54. L-Carnitine treatment reduces severity of physical and mental fatigue and increases cognitive functions in centenarians: a randomized and controlled clinical trialAm J Clin Nutr · PMID 18065594
  55. Effect of L-carnitine on exercise performance in patients with mitochondrial myopathyBraz J Med Biol Res · PMID 25714882
  56. The effect of l-carnitine on fat oxidation, protein turnover, and body composition in slightly overweight subjectsMetabolism · PMID 15281008
  57. Liver-Oriented Acute Metabolic Effects of A Low Dose of L-Carnitine under Fat-Mobilizing Conditions: Pilot Human Clinical TrialJ Nutr Sci Vitaminol · PMID 32350175
  58. Levocarnitine administration in elderly subjects with rapid muscle fatigue: effect on body composition, lipid profile and fatigueDrugs Aging · PMID 12875611

Supplement stack for Mitochondrial Myopathy