Protocol · Metabolic/Genetic Disorders
Mitochondrial Disease Supportive Care Protocol
Coenzyme Q10 and Riboflavin (Vitamin B2) are the core of this stack, with 6 supporting supplements. Each row gives the amount the protocol lists and the grade our evidence database holds for that supplement in Mitochondrial Disease Supportive Care.
We are re-verifying the studies cited on protocol pages; only confirmed citations are shown.
The stack
8 supplements · 2 graded
| Grade | Supplement | Amount listed | Why it’s in the stack | Evidence on file |
|---|---|---|---|---|
| Primary stackThe core of this protocol | ||||
| Coenzyme Q10 | Amount listed: 100–600 mg daily (higher doses often used in mito disease) | Essential electron carrier in respiratory chain; may improve energy production; most studied supplement for mito disease
| Grade D for Exercise Tolerance2 studies · 30 people | |
| Riboflavin (Vitamin B2) | Amount listed: 100–400 mg daily | FAD precursor; essential for Complex I and II; may benefit specific mutations | Not graded yet | |
| Supporting stackListed as additions to the core | ||||
| L-Carnitine | Amount listed: 30–100 mg/kg/day in divided doses (typically 1–3 g daily) | Transports fatty acids into mitochondria; often deficient in mito disease; may help with energy and muscle symptoms | Not graded yet | |
| Alpha-Lipoic Acid | Amount listed: 300–600 mg daily | Antioxidant; cofactor for mitochondrial enzymes; may help with oxidative stress | Not graded yet | |
| Creatine | Amount listed: 5–10 g daily | Alternative energy substrate; may help buffer ATP in energy-deficient states
| Grade D for Aerobic Exercise Metrics2 studies · 7 people | |
| B-Complex Vitamins | Amount listed: High-potency B-complex daily (with extra B1, B2, B3) | Multiple B vitamins are mitochondrial cofactors; supports overall energy metabolism | Not graded yet | |
| Vitamin E | Amount listed: 400–800 IU daily | Antioxidant; helps protect mitochondrial membranes from oxidative damage | Not graded yet | |
| Thiamine (Vitamin B1) | Amount listed: 100–300 mg daily | Essential cofactor for pyruvate dehydrogenase and alpha-ketoglutarate dehydrogenase | Not graded yet | |
How this protocol works
In plain language
Mitochondrial diseases are a group of disorders caused by dysfunctional mitochondria - the "powerhouses" of cells that produce energy (ATP). These conditions can be inherited or acquired and affect multiple organ systems, particularly those with high energy demands (brain, muscles, heart).
COMMON PRESENTATIONS:
- MELAS: Mitochondrial encephalomyopathy, lactic acidosis, stroke-like episodes
- MERRF: Myoclonic epilepsy with ragged red fibers
- LHON: Leber hereditary optic neuropathy
- Kearns-Sayre: External ophthalmoplegia, heart block, retinitis pigmentosa
- Leigh Syndrome: Progressive neurodegeneration in infants/children
COMMON SYMPTOMS:
- Exercise intolerance and fatigue
- Muscle weakness and pain
- Neurological problems (seizures, stroke-like episodes, developmental delay)
- Vision and hearing loss
- Heart problems
- Diabetes
- GI symptoms
CRITICAL: Mitochondrial diseases require specialist care. This protocol is SUPPORTIVE ONLY.
GENERAL MANAGEMENT:
- Avoid metabolic stress (fasting, extreme temperatures, illness)
- Treat infections promptly
- Avoid mitochondrial toxins (certain drugs, alcohol)
- Physical therapy and exercise (supervised, graded)
- Symptom management
DRUGS TO AVOID:
- Valproic acid
- Statins (caution)
- Aminoglycosides
- Metformin (caution in some)
- Propofol (caution)
CoQ10* is the most studied supplement and supports electron transport.
Riboflavin* is particularly important for Complex I deficiencies.
L-Carnitine* helps with fatty acid transport and is often low.
Expected timeline: Supplements may provide modest symptom improvement over weeks to months. Disease progression is variable.