Ask the Mito Doc – October 2023; Q&A

Ask the Mito Doc October 2023

Diet, Nutrition and Nutraceuticals for the Mitochondrial Disease Patient


Clinician: Dr. Mark Tarnopolsky, MD, PhD, FRCP(C), McMaster University Medical Center, Ontario, Canada


Q: Are there different types of Creatine? Does one type work better than the other, and what is the usual dosing in the mito cocktail for creatine?

A: There are so many myths about creatine. First and foremost, creatine monohydrate. Do not buy any other type other than creatine monohydrate. All of the pre-clinical work, 99% of clinical trials, were done with creatine monohydrate. Number 2, never ever buy liquid. Next, with creatine, don’t load. In the old days, athletes used to take 20 grams, which is almost 3 tablespoons a day. You do not need that. Studies have shown clearly that you can load the brain and muscle with a lower, constant dose. For most adults, one heaping teaspoon a day is sufficient, and for children, it’s a hundred milligrams per kilogram per day. And generally with creatine, what we want to do is mix it into applesauce or yogurt, essentially it’s just a white powder, it does not dissolve, so put it into something that’s semi viscous and divide it into doses, it’s got a very long half-life.


Q: Is there something specific about creatine for vegans? Do they need a higher dose?

A: Vegans get 0 from their diet and we know from their muscle biopsies that their muscles are about 30% lower in creatine concentration. Vegans don’t need any more than what I’ve suggested, just stick to the same doses. All I would say is, that if you’re vegan, you will get much more bang from your buck because the more important thing is replacing a deficiency, which is usually much more impactful than trying to super compensate a sufficient state.


Q: Regarding supplements, how would you approach finding the right supplement? Where do you buy it? What kinds are more bioavailable? What kinds are to be avoided?

A: I would say first, if it’s too good to be true, it probably is. Make sure that supplements that you take have research published on platforms like Pubmed. Also, consider the reputation of the company that manufactures the supplements. Reputable and larger companies often source their products from trustworthy suppliers. Always start with a low dose and gradually increase it to allow your body to adjust. In Canada, they’ve introduced an Npn (Natural Product Number) system for supplements. If a supplement has an Npn, it means it’s gone through strict guidelines for purity and quality. In the US, consider buying from reputable companies with good buying power. Stores like Costco, known for their quality standards, are reasonable places for supplements. Costco has incredible buying power.


Q: Is Keto a good diet for mitochondrial disease? If not what is the best diet or way of eating that is the best?

A: Only if PDH or uncontrolled seizures – try a paleo diet instead – easier to do and healthy.


Q: I’ve read Taurine has many protective effects for the eyes. Is that something you would recommend taking for eye protection?

A: Best evidence is for MELAS, but no harm – I suggest 500 mg twice a day.


Q: In the mito cocktail recommendations you list Vitamin E. Would it be better to take pure vitamin E tocotrienols vs regular tocopherol blends? Similarly, would you recommend CoQ10 vs Idebenone?

A: All I can say is that in our RCT we found that the ALA, CoQ10, vitamin E (alpha-tocopherol) + creatine lowered 2 markers of oxidative stress and lactate. VERY high CoQ10 alone did not.


Q: Would you address the supplementation that those of us with a cow’s milk allergy might want to add to our daily diet to compensate for this?

A: A cow milk allergy is VERY rare – most people have lactose intolerance – a proper whey and caseine isolate has the same lactose as lactose free milk.


Q: Is there a difference in the recommended dose of CoQ10 vs Q-Sorb?

A: We find similar effects – 200 mg twice a day for adults and 10 mg/kg in divided doses for kids – Q-Sorb is a fine brand but so are others like Kaneka.


Q: If you have high creatinine levels should you stay away from taking creatine?

A: Creatine is converted to creatinine – it will make numbers look higher by about 10 – 15 % BUT does not reflect kidney damage when you give the precursor to creatinine – most doctors do not get the biology or studies.


Q: Is a gluten free diet beneficial for Mito patients?

A: Only for celiac.


Q: Is there anything that may support glutamic acid and taurine?

A: Taurine – 100 % for MELAS tRNA mutations (high dose study done) – likely fine for everyone 500 mg twice a day.


Q: Regarding Alpha lipoic acid, I thought that went out of fashion a few years ago….mine was stopped. Should I re-start?

A: Makes no sense – see our paper – first RCT – in our RCT we found that the ALA, COQ10, vitamin E (alpha-tocopherol) + creatine lowered 2 markers of oxidative stress and lactate.


Q: I’ve heard that folinic acid is better than folic acid, if one has MTHFR deficiency (difficulty metabolizing folate). Would you agree?

A: Yes in that case, but much more expensive.


Q: Would there ever be a pediatric dosage available for Trim7?

A: Dose is for a 70 kg person – can relatively scale back since full dose is 6 pills.


Q: Is Trim7 available over the counter?

A: Only online now –


Q: In regards to underweight children, instead of a G tube is there another alternative such as medication that increases appetite or high calorie drink diet?

A: Sure – try frequent feeds and good quality protein.


Q: How can exercise supplement the dietary recommendations that you are making? How should patients balance maintaining muscle tone and fitness while limiting the exhaustion that comes with having a mito disease? How much exercise is recommended for mito patients?

A: Look at our mitochondrial patient information on YouTube:


Q: How can we share best practice in these established and emerging options around the world? Many patients and their (often busy and non-mito specializing) clinicians are struggling to make evidence informed and personalized decisions about which supplements to take. It appears that your center has some great insights, but I think we can do more to make this knowledge available to more people (patients and their clinician).

A: There are many options, but wait till it’s shown in a clinical trial to show safety and efficacy – we are very confident in safety and biomarker efficacy of ALA, COQ10, vitamin E (alpha-tocopherol) + creatine lowered 2 markers of oxidative stress and lactate.


Q: What suggestions would you make to improve disease management with respect to the process of prioritizing nutritional and other lifestyle factors to focus on and how to isolate, test and tweak them?

A: SBMA is not a mitochondrial disease BUT treat low testosterone – exercise (see our videos on YOUTUBE – type in my name and neuromuscular). Creatine would for sure be useful – can send you our creatine information sheet, email me at


Q: Given that you believe sun exposure is a risk factor for LHON, would you recommend supplements such as astaxanthin, or zeaxanthin & Lutein, which have been shown to provide UV protection? Would your protocol change for a LHON patient who has been affected for about two years, but still has one unaffected eye?

A: Yes – send me an email for my LHON information


Q: Mitochondrial disease is not an absolute contraindication for organ donation, is it?

A: Nope – not for CPEO.


Q: What about Creapure (made in Germany) vs regular Crmonohydrate?

A: We only use CREAPURE in our products – GMP facility.


Q23: Which kind of doctor should I be seeing for Carnitine Deficiency?

A: Metabolic specialist