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Order Code Q10 Coenzyme Q10, Reduced and Total, Plasma

Reporting Name

Coenzyme Q10, Reduced and Total, P

Useful For

Diagnosis of primary coenzyme Q10 (CoQ10) deficiencies in some patients who are not supplemented with CoQ10

 

Diagnosis of CoQ10 deficiency in mitochondrial disorders

 

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

 

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

Performing Laboratory

Mayo Clinic Laboratories in Rochester

Specimen Type

Plasma Heparin


Ordering Guidance


This test provides both reduced and total coenzyme Q10 (CoQ10). For assessment of total only, order TQ10 / Coenzyme Q10, Total, Plasma.

 

The level of oxidized CoQ10 is 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 (ie, 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.



Necessary Information


Patient's age is required.



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, aliquot plasma into plastic vial, and freeze immediately.


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

COENZYME Q10 (CoQ10) REDUCED

<18 years: 320-1376 mcg/L

≥18 years: 415-1480 mcg/L

 

CoQ10 TOTAL

<18 years: 320-1558 mcg/L

≥18 years: 433-1532 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

Day(s) Performed

Monday through Friday

CPT Code Information

82542

Method Description

Coenzyme Q10 (CoQ10) is extracted from plasma with cold 1-propanol containing coenzyme Q9 as an internal standard. An aliquot of the lipid extract is fractionated by high-performance liquid chromatography (HPLC). The separated coenzyme Q10 reduced and coenzyme Q10 oxidized are quantified by measurement of selective coulometric response emerging from the chromatographic column.(Tang PH, Miles MV, DeGrauw A, Hershey A, Pesce A. HPLC analysis of reduced and oxidized coenzyme Q[10] in human plasma. Clin Chem. 2001;47[2]:256-265; Claessens AJ, Yeung CK, Risler LJ, Phillips BR, Himmelfarb J, Shen DD. Rapid and sensitive analysis of reduced and oxidized coenzyme Q10 in human plasma by ultra performance liquid chromatography-tandem mass spectrometry and application to studies in healthy human subjects. Ann Clin Biochem. 2016;53[Pt 2]:265-273. doi:10.1177/0004563215593097)

Reject Due To

Gross hemolysis Reject
Gross lipemia Reject
Gross icterus OK

Method Name

High-Performance Liquid Chromatography (HPLC) with Electrochemical Detection

Genetics Test Information

This test is appropriate for the diagnosis of secondary coenzyme Q10 (CoQ10) deficiency and for 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.