When Are CoQ10 Blood Levels Useless?

Whenever they are normal or high, and here's why.

As I covered in Does CoQ10 Deserve a Spot in Your Longevity Plan?, no one should need a supplement of CoQ10 unless they have a genetic impairment or nutrient deficiency hurting its synthesis.

Many people might think that a good way to know if you need more is if your blood levels are low, and that if they’re not, you don’t need more.

This is not true. I use the Genova ION + 40 as one of the most important nutritional testing panels, and it includes serum CoQ10. I also use the Vibrant America Micronutrient Panel, which includes both serum and white blood cell CoQ10. In my experience, high CoQ10 on Genova often but not always is accompanied by low or low-normal white blood cell CoQ10 on Vibrant, with the Vibrant serum being high or high normal like the Genova measurement.

These are likely cases of poor CoQ10 transport into cells, for the reasons discussed in Does CoQ10 Deserve a Spot in Your Longevity Plan?, but you would be deeply misled if you had only run the Genova panel.

(Note, I use these panels in the context of the broader Comprehensive Nutritional Screening).

This is educational in nature and not medical or dietetic advice. See terms for additional and more complete disclaimers.

We can put aside transport problems and just consider synthesis problems. You would think if you weren’t synthesizing CoQ10 sufficiently the blood levels would be low, yet this is falsified by the normal blood levels that occur in many CoQ10 synthesis disorders where biopsies are needed to find the low CoQ10 levels.

For example, CoQ8A patients usually show low CoQ10 in muscle but not skin fibroblasts, and serum levels have not been reported. Serum levels probably haven’t been reported because previous experience with other CoQ10 synthesis disorders showed they were useless.

In patients with another CoQ10 synthesis defect, PDDS1, serum levels are usually normal despite deficits in skin fibroblasts.

In the case of both mutations, exercise intolerance and neurological problems such as ataxia (difficulty coordinating movements) and tremors respond to CoQ10 supplementation, something that was not predicted by serum levels but was predicted by biopsies of the correct tissue. But even in biopsied tissue, the fibroblasts work in PDDS1 mutations but not in CoQ8A mutations, so “just biopsy the tissue” doesn’t work either.

This is probably because different mutations have greater impacts in some tissues than others. It may also be that some tissues get preference over what can be synthesized, so different severities of different mutations might determine whether, say, skin fibroblasts display low levels. And it’s probably also influenced by the bias of which very small numbers of patients were found with which mutations.

We know very little about how CoQ10 is transported, and almost nothing about how the burden of synthesis is shared by different tissues. For example, recent reviews suggest that it is probably the case that the liver synthesizes more than its own share and plays a role in distributing it other tissues, but this is based mostly on the liver doing this regularly with many other things and not on rigorous data about the liver doing this with CoQ10.

The point is that you can need CoQ10 to such a degree it is giving you neurological problems and exercise intolerance and your blood levels could be normal or even high.

In a related matter, I have recently discovered that pathogenic mutations in ANO10 can cause CoQ10 deficiency. This has not been mentioned in any of the reviews I have read about CoQ10 synthesis and transport, but I recently found a 2014 paper showing this is the case.

The exact role of ANO10 is not well understood. It is thought to be a chloride channel but we are pretty clueless about its physiological function. In two patients, mutations in this gene have been tied to CoQ10 deficiency.

One patient developed epilepsy at age 7 that was well-controlled through adulthood and then from the ages of 45-50 developed gait disturbance, slurred speech, and ataxia, leading her to be wheelchair-bound. Muscle biopsy showed low CoQ10. Supplementation with 1000 milligrams per day of CoQ10 abolished the epilepsy and within 3-4 months improved the fatigue, mobility, cognition, and the ability to walk.

The second patient was normal until age 30, then developed a very slowly progressing difficulty walking and slurred speech. By the age of 50 she was using a walker because she had suffered so many injuries from falling. She also suffered from retinal detachment and intention tremor. CoQ10 levels were normal in skin biopsy, borderline low in muscle, and low in plasma. 120-180 milligrams of CoQ10 per day mitigated these problems. 

Notice that the second patient showed the opposite pattern as the previous mutations mentioned, with plasma having the greatest signal and muscle the least.

That doesn’t mean everyone should just willy nilly supplement with CoQ10. As I covered in Does CoQ10 Deserve a Spot in Your Longevity Plan?, in subjects with Parkinson’s, high-dose CoQ10 appeared to cause such problems as orthostatic hypotension, acid reflux, and heart palpitations, there’s an unclearly described case series seeming to suggest it increased the risk of death in ALS patients, and in mice it worsens the age-related loss of hearing and spatial learning and memory.

My recommendations for CoQ10 supplementation are covered in Does CoQ10 Deserve a Spot in Your Longevity Plan?

My point here is simple: never dismiss the possibility you might need CoQ10 based on normal or high serum levels.