25000 Coenzyme Q10, Reduced and Total, Plasma (Q10)

Diagnosis of primary CoQ10 deficiencies in some patients who are not supplemented with CoQ10

Diagnosis of coenzyme Q10 (CoQ10) deficiency in mitochondrial disorders

Monitoring CoQ10 status during treatment of various degenerative conditions including Parkinson and Alzheimer disease

This test is not useful for distinguishing primary CoQ10 deficiencies from acquired CoQ10 deficiencies.

This test is appropriate for the diagnosis of secondary coenzyme Q10 (CoQ10) deficiency, and in some patients with primary CoQ10 deficiency who are not supplemented with CoQ10. It is also used to monitor CoQ10 status in patients with mitochondrial cytopathies, patients receiving statin therapy, or during treatment of various degenerative conditions including Parkinson and Alzheimer diseases.

1. Immediately after collection, place specimen on wet ice. Maintain on wet ice during processing. 

2. Within 3 hours of collection, centrifuge, aliquot plasma into a plastic vial, and freeze immediately​.

If possible, DO NOT send other tests ordered on same vial of plasma. In doing so, the other tests may have increased turnaround time due to the strict frozen criteria of this assay.

​Coenzyme Q10 (CoQ10) is sensitive to specimen handling and transport temperature. Failure to follow the specimen handling and transportation recommendations may lead to false-positive results.

Coenzyme Q10 (CoQ10) is an essential cofactor in the mitochondrial respiratory chain responsible for oxidative phosphorylation where it functions as an electron carrier and acts as an antioxidant. It is found in all cell membranes and is carried by lipoproteins in the circulation. Approximately 60% of CoQ10 is associated with low-density lipoprotein (LDL), 25% with high-density lipoprotein, and 15% with other lipoproteins. CoQ10 is present in the body in both the reduced and oxidized forms, with the antioxidant activity of CoQ10 dependent on both its concentration and its reduction-oxidation (redox) status.

Coenzyme Q10 deficiencies, which are clinically and genetically diverse, can occur due to defects in genes involved in the biosynthesis of ubiquinone (primary CoQ10 deficiency) or due to other causes, such as mitochondrial disorders (secondary or CoQ10 deficiency).

Five major clinical phenotypes of CoQ10 deficiency have been described:

-Encephalomyopathy (elevated serum creatine kinase [CK], recurrent myoglobinuria, lactic acidosis)

-Cerebellar ataxia and atrophy (neuropathy, hypogonadism)

-Severe multisystemic infant form (nystagmus, optic atrophy, sensorineural hearing loss, dystonia, rapidly progressing nephropathy)

-Nephropathy, steroid resistant nephrotic syndrome leading to end stage kidney disease

-Isolated myopathy (exercise intolerance, fatigue, elevated serum CK)

Treatment with CoQ10 in patients with mitochondrial cytopathies can improve mitochondrial respiration in both brain and skeletal muscle.

​Coenzyme Q10 has been implicated in other disease processes, including diabetes, neurodegenerative conditions such as Parkinson and Alzheimer diseases, as well as in aging and oxidative stress. CoQ10 may also play a role in hydroxymethylglutaryl-CoA reductase inhibitor (statin) therapy and may be relevant to statin-induced myalgia. Additionally, the redox status of CoQ10 may be a useful early marker for the detection of oxidative LDL modification.

Abnormal results are reported with a detailed interpretation including an overview of the results and their significance, a correlation to available clinical information provided with the specimen, differential diagnosis, and recommendations for additional testing when indicated and available. ​