Condition

Mitochondrial Cytopathy

Mitochondrial cytopathy is a group of disorders in which mitochondria, the tiny structures that make cellular energy, work poorly, mostly affecting the brain, muscles and heart. Coenzyme Q10 is the most studied supplement for it, alongside creatine, but trials found no clear difference in exercise tolerance or aerobic exercise measures. Those studies were very small, about 30 people for coenzyme Q10 and just 7 for creatine, so the evidence is very limited.

Sources: PMID 20886510; PMID 9390662

Supplements studied: 2
Graded outcomes: 2
Trials: 2
People studied: 37

Evidence by supplement

Strong
Moderate
Limited
Very limited

2 supplements

Coenzyme Q10

Coenzyme Q10: outcomes studied for Mitochondrial Cytopathy
Grade	Outcome	Size	Studies	People
	Exercise Tolerance No effect	NoneNo effect	2 studies	30 people
Studies that measured exercise tolerance Coenzyme Q10 terclatrate and creatine in chronic heart failure: a randomized, placebo-controlled, double-blind studyClin Cardiol · PMID 21462215 A randomized trial of coenzyme Q10 in mitochondrial disordersMuscle Nerve · PMID 20886510
	Quality of Life No effect		1 study
Studies that measured quality of life A randomized trial of coenzyme Q10 in mitochondrial disordersMuscle Nerve · PMID 20886510

Creatine

Creatine: outcomes studied for Mitochondrial Cytopathy
Grade	Outcome	Effect	Size	Studies	People
	Aerobic Exercise Metrics No effect		NoneNo effect	2 studies	7 people
Studies that measured aerobic exercise metrics Creatine supplementation does not enhance submaximal aerobic training adaptations in healthy young men and womenEur J Appl Physiol · PMID 16896727 A randomized, controlled trial of creatine monohydrate in patients with mitochondrial cytopathiesMuscle Nerve · PMID 9390662
	Mitochondrial Cytopathies Symptoms	Improves (the measure goes down)		1 study	7 people
Studies that measured mitochondrial cytopathies symptoms Supplementation with creatine monohydrate in children with mitochondrial encephalomyopathiesMuscle Nerve · PMID 10454733
	Fat Oxidation	Improves (the measure goes up)			7 people
	Oxygen Uptake
	Power Output

Key findings

Exercise ToleranceCoenzyme Q10
Aerobic Exercise MetricsCreatine

The Mitochondrial Disease Supportive Care protocol

2 primary supplements · 6 supporting supplements

Coenzyme Q10100–600 mg daily (higher doses often used in mito disease)
Riboflavin (Vitamin B2)100–400 mg daily

See the full protocol

Studies cited

5 studies from PubMed

A randomized, controlled trial of creatine monohydrate in patients with mitochondrial cytopathiesMuscle Nerve · PMID 9390662
Supplementation with creatine monohydrate in children with mitochondrial encephalomyopathiesMuscle Nerve · PMID 10454733
Creatine supplementation does not enhance submaximal aerobic training adaptations in healthy young men and womenEur J Appl Physiol · PMID 16896727
A randomized trial of coenzyme Q10 in mitochondrial disordersMuscle Nerve · PMID 20886510
Coenzyme Q10 terclatrate and creatine in chronic heart failure: a randomized, placebo-controlled, double-blind studyClin Cardiol · PMID 21462215