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What Is CoQ10 (coenzyme Q10)?

January 2012 — In response to recent statements and discussion regarding CoQ10 on the NPC listserv, we have the following update: 

Oxidative Stress in Niemann-Pick disease, type C
Authors: Rao Fu, Nicole M. Yanjanin, Simona Bianconi, William J. Pavan and Forbes D. Porter

Dr. Forbes “Denny” Porter pulled pertinent data from the study, below:

Reduced CoQ10 fraction was significantly decreased in NPC patients. Serum CoQ10 levels were measured in 32 NPC subjects during an evaluation when they were not supplemented with either CoQ10 or antioxidants. Initial levels for reduced and total CoQ10 are shown in Fig. 1A. For the 28 NPC patients less than 18 years of age, the mean reduced and total CoQ10 levels were 371±20 mcg/L and 428±24 mcg/L, respectively, both values being within the reference range. Only four (14%) pediatric NPC patients had a total CoQ10 level below the lower reference range.

These data demonstrate that a CoQ10 deficiency is not a common finding in NPC patients. However, the fraction of reduced CoQ10 is decreased in NPC patients (Fig. 1B).

The mean fraction of reduced CoQ10 was 86.8±1.6%, which is much less than the lower limit of the reference range (93%). Twenty-two patients (79%) had reduced CoQ10 fractions below 93%. Similar results were obtained for four NPC patients 18 years of age and older. Mean reduced and total CoQ10 levels were 617±224 mcg/L and 674±233 mcg/L (Fig.1A), and the fraction of reduced CoQ10 was decreased (Fig. 1B). There was no correlation of CoQ10 levels with either disease severity or age of onset (data not shown).

Supplementation of CoQ10 does not correct the abnormal fraction of reduced CoQ10. For patients receiving CoQ10 supplementation, we analyzed both initial and serial values. Five patients were on supplementation at their initial evaluation, and two patients were supplemented after their initial evaluation. A total of nine evaluations of patients on CoQ10 supplementation were available.

As one would predict, samples obtained from CoQ10 supplemented patients had significantly higher levels of both reduced (pb.0001) and total CoQ10 (pb.0001) compared to unsupplemented patients (Fig. 1C). Reduced CoQ10 levels were increased 94%, and total levels were increased 128%. However, supplementation failed to correct the abnormal fraction of reduced CoQ10 (Fig. 1D).

The fraction of reduced CoQ10 in supplemented patients (86.6±2.7%) was not significantly different (p=.64) than that observed in unsupplemented samples (87.6±9.2%). We also evaluated the effect of miglustat therapy on CoQ10 levels. Mean reduced and total CoQ10 levels were decreased in miglustat treated patients (Fig. 2A); however, miglustat therapy had no effect on the fraction of reduced CoQ10 (Fig. 2B). Consistent with this latter observation, no differences were seen in the pre- and post-miglustat
treatment CoQ10 values for five patients (data not shown).

[Jan 9, 2012 mem]

This page is not intended as medical advice. Consult with your physician before undertaking any treatment or therapy.

What is CoQ10?

CoQ10 or coenzyme 10 is a nutritional supplement. The enzyme acts as an anti-oxidant to neutralize free radicals which can damage cells. It has been shown to help prevent heart disease (and is widely prescribed in Japan for this) and there is evidence that it improves neurological functions. CoQ10 also acts to lower blood cholesterol levels.

Only a limited number of research studies have been done on Coenzyme Q10 and many of these are related to heart disease. Dr. Salvatore DiMauro, of Columbia University, conducted one study on hereditary ataxia and CoQ10. Hereditary ataxia affects coordination, balance, and speech. Test subjects showed improvement, with some being able to leave wheelchairs and begin using walkers. However, the number of patients in the study was so small that no definite conclusions were possible.

No controlled studies have been done on the effect of Coenzyme Q10 on Niemann-Pick Type C individuals.

Blood samples from several NPC individuals were taken. These samples were analyzed by Dr. DiMauro and Dr. Marc Patterson to determine if a deficiency of CoQ10 might exist with NP Type C. See a report of those studies in the Spring 2003 NNPDF Newsletter.

Is CoQ10 safe?

There are no side effects reported in medical literature from using CoQ10 by itself. However, the number of studies is limited. Some families have reported diarrhea as a problem when using the powder form. It has been reported that there can be negative effects if CoQ10 is stopped quickly.

Nutritional supplements are not prescribed or regulated in the United States even when they have medicinal qualities. Some supplements can interact with other medications and some have maximum safe dosages. Certain formulations of CoQ10 have other supplements added, which may have their own side effects. You should consult with your physician before starting your child on CoQ10 or any other supplement or therapy.