Test Id : Q10
Coenzyme Q10, Reduced and Total, Plasma
Useful For
Suggests clinical disorders or settings where the test may be helpful
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.
Genetics Test Information
Provides information that may help with selection of the correct genetic test or proper submission of the test request
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.
Method Name
A short description of the method used to perform the test
High-Performance Liquid Chromatography (HPLC) with Electrochemical Detection
NY State Available
Indicates the status of NY State approval and if the test is orderable for NY State clients.
Reporting Name
Lists a shorter or abbreviated version of the Published Name for a test
Specimen Type
Describes the specimen type validated for testing
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
Defines the optimal specimen required to perform the test and the preferred volume to complete testing
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.
Forms
If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.
Specimen Minimum Volume
Defines the amount of sample necessary to provide a clinically relevant result as determined by the testing laboratory. The minimum volume is sufficient for one attempt at testing.
0.3 mL
Reject Due To
Identifies specimen types and conditions that may cause the specimen to be rejected
| Gross hemolysis | Reject |
| Gross lipemia | Reject |
| Gross icterus | OK |
Specimen Stability Information
Provides a description of the temperatures required to transport a specimen to the performing laboratory, alternate acceptable temperatures are also included
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Plasma Heparin | Frozen (preferred) | 14 days | |
| Refrigerated | 8 hours |
Useful For
Suggests clinical disorders or settings where the test may be helpful
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.
Genetics Test Information
Provides information that may help with selection of the correct genetic test or proper submission of the test request
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.
Clinical Information
Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test
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.
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)
-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.
CoQ10 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.
Reference Values
Describes reference intervals and additional information for interpretation of test results. May include intervals based on age and sex when appropriate. Intervals are Mayo-derived, unless otherwise designated. If an interpretive report is provided, the reference value field will state this.
COENZYME Q10 (CoQ10) REDUCED
<18 years: 320-1376 mcg/L
> or =18 years: 415-1480 mcg/L
CoQ10 TOTAL
<18 years: 320-1558 mcg/L
> or =18 years: 433-1532 mcg/L
CoQ10 % REDUCED
<18 years: 93-100%
> or =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
Interpretation
Provides information to assist in interpretation of the test results
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.
Cautions
Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
Coenzyme Q10 is sensitive to specimen handling and transport temperature. Failure to follow the specimen handling and transportation recommendations may lead to false-positive results.
Clinical Reference
Recommendations for in-depth reading of a clinical nature
1. Salviati L, Trevisson E, Agosto C, Doimo M, Navas P. Primary coenzyme Q10 deficiency overview. In: Adam MP, Mirzaa GM, Pagon RA, et al. eds. GeneReviews [Internet]. University of Washington, Seattle; 2017. Updated June 8, 2023. Accessed November 1, 2023. Available at www.ncbi.nlm.nih.gov/books/NBK410087/
2. Desbats MA, Lunardi G, Doimo M, Trevisson E, Salviati L. Genetic bases and clinical manifestations of coenzyme Q10 (CoQ 10) deficiency. J Inherit Metab Dis. 2015;38(1):145-56. doi:10.1007/s10545-014-9749-9
3. Littarru GP, Tiano L. Clinical aspects of coenzyme Q10: An update. Nutrition. 2010;26:250-254
4. Hargreaves I, Heaton RA, Mantle D. Disorders of human coenzyme Q10 metabolism: An overview. Int J Mol Sci. 2020;21(18):6695. doi:10.3390/ijms21186695
5. Banach M, Serban C, Ursoniu S, et al. Statin therapy and plasma coenzyme Q10 concentrations-A systematic review and meta-analysis of placebo-controlled trials. Pharmacol Res. 2015;99:329-336. doi:10.1016/j.phrs.2015.07.008
6. Emmanuele V, Lopez LC, Berardo A, et al. Heterogeneity of coenzyme Q10 deficiency: patient study and literature review. Arch Neurol. 2012;69(8):978-983. doi:10.1001/archneurol.2012.206
Method Description
Describes how the test is performed and provides a method-specific reference
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)
PDF Report
Indicates whether the report includes an additional document with charts, images or other enriched information
Day(s) Performed
Outlines the days the test is performed. This field reflects the day that the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time before the test is performed. Some tests are listed as continuously performed, which means that assays are performed multiple times during the day.
Monday through Friday
Report Available
The interval of time (receipt of sample at Mayo Clinic Laboratories to results available) taking into account standard setup days and weekends. The first day is the time that it typically takes for a result to be available. The last day is the time it might take, accounting for any necessary repeated testing.
Specimen Retention Time
Outlines the length of time after testing that a specimen is kept in the laboratory before it is discarded
Performing Laboratory Location
Indicates the location of the laboratory that performs the test
Fees :
Several factors determine the fee charged to perform a test. Contact your U.S. or International Regional Manager for information about establishing a fee schedule or to learn more about resources to optimize test selection.
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Test Classification
Provides information regarding the medical device classification for laboratory test kits and reagents. Tests may be classified as cleared or approved by the US Food and Drug Administration (FDA) and used per manufacturer instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR) product.
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.
CPT Code Information
Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Clinic Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.
CPT codes are provided by the performing laboratory.
CPT codes are provided by the performing laboratory.
82542
LOINC® Information
Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the order and results codes of this test. LOINC values are provided by the performing laboratory.
| 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
Applies only to results expressed in units of measure originally reported by the performing laboratory. These values do not apply to results that are converted to other units of measure.
|
|---|---|---|
| 87853 | CoQ10 reduced | 81157-0 |
| 30091 | CoQ10 Total | 27923-2 |
| 30092 | CoQ10 % reduced | 81156-2 |
| 30159 | Interpretation | 59462-2 |