There have been very few options available for treating and managing mitochondrial disease and dysfunction (both of which are referred to as "mito"). As a result, this is one area of conventional medicine in which the value of nutritional supplements has been accepted and embraced. People with mito usually take a custom blend of supplements every day that is refereed to as a "mito cocktail". Many people try various combinations of these vitamins and supplements on themselves to see what helps, especially those with mito dysfunction (which is generally acquired from toxic injury, is generally much more mild, and much harder to find a doctor willing or able to treat). There are some general guidelines and some basic components that are safe, so it is possible to go about it this way, but the doses of the components may need to be very high and it can be very hard for individuals to get the doses and combination right for their needs. However, since so few people have access to doctors to treat mito, this is the best that many people can do. Once a good "cocktail" is developed for a patient they may choose to have it compounded, which can make the cocktail much easier to take (especially if
it includes many different components).
The Mito cocktail explained from Mito Action
Diagnosis and management of patients with mitochondrial disease (specifically table 2)
Letter explaining importance and cost benefit of mito cocktail
The Effect of Mitochondrial Supplements on Mitochondrial Activity in Children with Autism Spectrum Disorder
The Basic Components:
Many people include Alpha Lipoic Acid (ALA) in the mito cocktail, and while it is supportive of mito function it is also a powerful chelator of mercury, lead, and other toxic metals and can cross the blood-brain-barrier so if there is a body burden of toxic metals it can push those metals into the brain. Use this with caution if metal toxicity is known or suspected.
Antioxidants - these are plant chemicals that protect the mitochondria from damage caused by oxidative stress. Oxidative stress produces "free radicals" as a byproduct of energy production which can harm cells. More on what free radicals are from the NIH site Office of Dietary Supplements:
"An atom or molecule made in the body that can damage cells. A free radical has at least one unpaired electron, which makes it unstable. To become stable, the free radical takes an electron away from another atom, which makes that atom unstable, and starts a chain reaction that can injure cells. Free radicals are made during chemical changes that take place in a cell or an organism to produce energy and basic materials needed for important life processes (metabolism). They also come from tobacco smoke, pollution, radiation from the sun and x-rays, and other sources outside the body. Free radicals damage cells, cause genetic alterations (mutations), and may play a role in cancer, heart disease, and age-related diseases (such as Alzheimer's, Parkinson's, and Lou Gehrig's diseases). Free radicals are also beneficial; they are involved in killing germs (microorganisms) and they help hormones and chemical messengers communicate with cells. Proteins (enzymes) made by the body, and vitamin C, vitamin E, and beta carotene in the diet help prevent free radical damage."
Arginine - is another amino acid that can be very helpful for some people with mito, especially the form called MELAS which involves stroke-like episodes. Arginine usually needs to be taken in very high doses. Arginine and another amino acid Lysine need to be consumed in balance for those who have chronic problems with herpes viruses (this includes cold sores, genital herpes, chicken pox/shingles, Epstein-Barr, Cytomegalovirus, and HHV6).
B vitamins - the B vitamins, especially thiamine (B1) and
Riboflavin (B2) are cofactors needed for the electron transport chain to
function. Riboflavin (B2) has shown to be particularly helpful for
people with headaches and migraines.
Folic Acid and Folate - (often considered to be a B vitamin) is also important.
L-Carnitine - is an amino acid that helps to transport fat into
the mitochondria so that it can be burned for fuel. This amino acid is
very common in the diet so there is rarely a deficiency, however high
doses can be quite therapeutic for people with mito. If buying this as a
supplement, acetyl-l-carnitine is usually the preferred form,
however the levocarnitine form is often considered best and is available
by prescription.
CoQ10 - this is an antioxidant found in almost all cells in the body. It plays an important role in transporting one of the important pieces of the electron transport chain, which is the machinery in the mitochondria that converts food and oxygen into energy that our bodies can use (ATP). It is much better absorbed if taken with an oil or fat-containing food. More on CoQ10 can be found here at the University of Maryland Medical Center site, although this information is more general and not about mito specifically.
D-Ribose - is a sugar that feeds the mitochondria.Enhancing Mitochondrial Function With D-Ribose
D-Ribose Helps People with Fibromyalgia and Chronic Fatigue Syndrome (CFS)
"D-Ribose is a naturally occurring simple sugar found in all living cells. It is the fuel that mitochondria use to produce adenosine tri-phosphate (ATP), which provides the body’s cellular energy. Studies show that patients with fibromyalgia, chronic fatigue syndrome (CFS) and congestive heart failure are low in D-Ribose."
"These Alzheimer's mice have extensive mitochondrial dysfunction, which likely contributes to their cognitive decline. To further explore the mechanism through which caffeine and melatonin protect cognitive function in these mice, we monitored the function of isolated mitochondria from APP(sw) mice treated with caffeine, melatonin, or both in their drinking water for one month. Melatonin treatment yielded a near complete restoration of mitochondrial function in assays of respiratory rate, membrane potential, reactive oxygen species production, and ATP levels. Caffeine treatment by itself yielded a small increase in mitochondrial function. However, caffeine largely blocked the large enhancement of mitochondrial function provided by melatonin."
Vitamin K - can increase availability of energy to mitochondria during exercise.
Low dose resveratrol ameliorates mitochondrial respiratory dysfunction and enhances cellular reprogramming
The optimum dosage is approximately 300-350 mg calcium as calcium citrate for a total of 1000 mg (one gram) of calcium a day. If you’re taking this you don’t need additional sources of calcium. An even better approach would be to use magnesium citrate. The adult dosage is about 300-400 mg a day. Some practitioners recommend up to 1000 mg but many people report problems with diarrhea if they exceed 400 mg. Again, a divided dose would be best, taking the magnesium citrate with each meal.
As an example, this formulation was made for a 3yo child:
CoQ10 180 mg,
Vit. E 450 iu,
Vit C 720 mg,
Carnitine 900 mg,
ALA 180 mg,
Biotin 2 mg,
Pantothenate 135 mg.