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Test ID: Q10 Coenzyme Q10, Reduced and Total, Plasma

Reporting Name

Coenzyme Q10, Reduced and Total, P

Useful For

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.

Clinical Information

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 (HDL), 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.

 

CoQ10 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)

-Glomerulopathy

-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.

 

CoQ10 has been implicated in other disease processes, including Parkinson disease, diabetes, and Alzheimer disease, as well as in aging and oxidative stress. CoQ10 may also play a role in hydroxymethylglutaryl-CoA reductase inhibitor (statin) therapy; changes in CoQ10 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.

Interpretation

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.

Analytic Time

3 days (not reported Saturday or Sunday)

Day(s) and Time(s) Performed

Tuesday, Friday

Clinical Reference

1. Salviati L, Trevisson E, Doimo M, et al: Primary Coenzyme Q10 Deficiency. In GeneReviews. Edited by MP Adam, HH Ardinger, RA Pagon, et al. University of Washington, Seattle. 1993-2019. 2017 Jan 26. Accessed 05/07/2019. Available at https://www.ncbi.nlm.nih.gov/books/NBK410087/

2. Desbats MA, Lunardi G, Doimo M, et al: Genetic bases and clinical manifestations of coenzyme Q10 (CoQ 10) deficiency. J Inherit Metab Dis 2015 Jan;38(1):145-156

3. Littarru GP, Tiano L: Clinical aspects of coenzyme Q10: An update. Nutrition 2010;26:250-254

4. Steele PE, Tang PH, DeGrauw AJ, Miles MV: Clinical laboratory monitoring of coenzyme Q10 use in neurologic and muscular diseases. Am J Clin Pathol 2004 June;121:S113-S120

5. Banach M, Serban C, Ursoniu S, et al; Lipid and Blood Pressure Meta-analysis Collaboration (LBPMC) Group. Statin therapy and plasma coenzyme Q10 concentrations-A systematic review and meta-analysis of placebo-controlled trials. Pharmacol Res 2015 Sep;99:329-336

Method Name

High-Performance Liquid Chromatography (HPLC) with Electrochemical Detection

Specimen Type

Plasma Heparin


Advisory Information


This test provides both reduced and total coenzyme Q10. For assessment of total only, order TQ10 / Coenzyme Q10, Total, Plasma.

 

The level of oxidized Q10 was affected in specimens with even slight amounts of hemolysis; however, the total Q10 level remains constant. Hemolyzed specimens can be analyzed for total Q10 using TQ10 / Coenzyme Q10, Total, Plasma.

 

The most reliable test for the diagnosis of primary defects in ubiquinone (CoQ10) biosynthesis is direct measurement of CoQ10 in muscle.



Shipping Instructions


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.



Specimen Required


Patient Preparation: Fasting (8 hours)

Collection Container/Tube: Green top (lithium or sodium heparin)

Submission Container/Tube: Plastic vial

Specimen Volume: 0.5 mL

Collection Instructions:

1. Immediately after collection, place specimen on wet ice. Maintain on wet ice and process within 3 hours of collection.

2. Centrifuge, separate plasma from cells, and immediately freeze specimen.


Specimen Minimum Volume

0.3 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Plasma Heparin Frozen (preferred) 14 days
  Refrigerated  8 hours

Reference Values

CoQ10 REDUCED

<18 years: 320-1,376 mcg/L

≥18 years: 415-1,480 mcg/L

 

CoQ10 TOTAL

<18 years: 320-1,558 mcg/L

≥18 years: 433-1,532 mcg/L

 

CoQ10 % REDUCED

<18 years: 93-100%

≥18 years: 92-98%

 

Miles MV, Horn PS, Tang PH, et al: Age-related changes in plasma coenzyme Q10 concentrations and redox state in apparently healthy children and adults. Clin Chim Acta 2004;34:139-144

Test Classification

This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the U.S. Food and Drug Administration.

CPT Code Information

82542

LOINC Code Information

Test ID Test Order Name Order LOINC Value
Q10 Coenzyme Q10, Reduced and Total, P In Process

 

Result ID Test Result Name Result LOINC Value
87853 CoQ10 reduced 81157-0
30091 CoQ10 Total 27923-2
30092 CoQ10 % reduced 81156-2
30159 Interpretation 59462-2

Forms

If not ordering electronically, complete, print, and send an Inborn Errors of Metabolism Test Request (T798) with the specimen.

Mayo Clinic Laboratories | Neurology Catalog Additional Information:

mml-Pediatric, mml-Neurometabolic