Coenzyme Q10 (CoQ10), also known as ubiquinone, is a cofactor, electron carrier, and antioxidant. It is a critical component of the electron transport chain in mitochondria. Inadequate amounts of CoQ10 lead to a failure of mitochondria to produce cellular energy. Coenzyme Q10 is found in all membranes and is carried by various cholesterol components in the bloodstream including low-density lipoprotein (LDL) and high-density lipoprotein (HDL). Total coenzyme Q10 is measured in hemolyzed blood, which means the blood cells are broken apart prior to analysis. The coenzyme Q10 test is primarily used to diagnose of coenzyme Q10 deficiency in mitochondrial disorders. It may also be used to monitor treatment of neurodegenerative diseases such as Alzheimer’s disease, Parkinson's disease and amyotrophic lateral sclerosis.
Coenzyme Q 10 deficiency may occur in three ways: primary coenzyme Q10 deficiency, secondary coenzyme Q10 deficiency, or related to another drug or disease. Primary coenzyme Q10 deficiency is rare and caused by genetic disorder that affects the CoQ10 molecule. Secondary coenzyme Q 10 deficiency, on the other hand, is caused by a problem in the mitochondria. Mitochondrial diseases cause a variety of symptoms but most are related to muscle weakness and neurological symptoms. This can lead to breathing problems, heart muscle problems, seizures, and cognitive problems.
Some specific causes of low coenzyme Q10 levels are:
- Mitochondrial diseases
- Primary CoQ10 deficiency (rare)
- Secondary CoQ10 deficiency
- Cerebellar ataxia
- Severe infantile multisystem disease
- Isolated myopathy
- Statin use
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Abnormally high levels of coenzyme Q10 are only practically possible through the use of supplements. It is unclear whether moderately excessive amounts of coenzyme Q10 are harmful to humans. While it is fat soluble, CoQ10 does not accumulate after supplementation has stopped.
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