Coenzyme Q10, Reduced and Total, Plasma
NY State Approved Indicates the status of NY State approval and if the test is orderable for NY State clients.
Diagnosis of coenzyme Q10 (CoQ10) deficiency in mitochondrial disorders
Monitoring patients receiving statin therapy
Monitoring CoQ10 status during treatment of various degenerative conditions including Parkinson and Alzheimer disease
Genetics Test Information Provides information that may help with selection of the correct test or proper submission of the test request
Diagnosis of coenzyme Q10 (CoQ10) deficiency in mitochondrial disorders. Monitoring coenzyme Q10 status in patients with mitochondrial cytopathies, patients receiving statin therapy, or during treatment of various degenerative conditions including Parkinson and Alzheimer diseases.
High-Performance Liquid Chromatography (HPLC) with Electrochemical Detection
Reporting Name A shorter/abbreviated version of the Published Name for a test; an abbreviated test name
Coenzyme Q10, Reduced and Total, P
Specimen Type Describes the specimen type needed for testing
Specimen Required Defines the optimal specimen. This field describes the type of specimen required to perform the test and the preferred volume to complete testing. The volume allows automated processing, fastest throughput and, when indicated, repeat or reflex testing.
Collection Container/Tube: Green top (lithium or sodium heparin)
Submission Container/Tube: Plastic vial
Specimen Volume: 0.5 mL
1. Fasting (8 hours)
2. Immediately place specimen on wet ice. Maintain on wet ice and process within 3 hours of draw.
3. Spin down, separate plasma from cells, and immediately freeze specimen.
Forms: If not ordering electronically, submit a Biochemical Genetics Request Form (Supply T439) with the specimen.
Specimen Minimum Volume Defines the amount of specimen required to perform an assay once, including instrument and container dead space. Submitting the minimum specimen volume makes it impossible to repeat the test or perform confirmatory or perform reflex testing. In some situations, a minimum specimen volume may result in a QNS (quantity not sufficient) result, requiring a second specimen to be collected.
Mild reject; Gross reject
Mild reject; Gross reject
Mild OK; Gross OK
Plasma gel tube, serum gel tube, EDTA plasma, or serum
Specimen Stability Information Provides a description of the temperatures required to transport a specimen to the laboratory. Alternate acceptable temperature(s) are also included.
|Plasma Heparin||Frozen (preferred)||72 hours|
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 also acts as an antioxidant. It is found in all cell membranes and 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 not only on its concentration, but also on its reduction-oxidation (redox) status.
Primary CoQ10 deficiency, although rare, is characterized by neurological symptoms (seizures, developmental delay, ataxia, etc) and muscle weakness. At least 5 different phenotypes have been described:
-Encephalomyopathy (elevated serum creatine kinase [CK], recurrent myoglobinuria, lactic acidosis)
-Childhood-onset cerebellar ataxia and atrophy (neuropathy, hypogonadism)
-Multisystemic infant form (nystagmus, optic atrophy, sensorineural hearing loss, dystonia, rapidly progressing nephropathy)
-Myopathy (exercise intolerance, fatigue, elevated serum CK)
Treatment with CoQ10 in patients with mitochondrial cytopathies improves 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. 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.
<18 years: 320-1,376 mcg/L
> or =18 years: 415-1,480 mcg/L
<18 years: 320-1,558 mcg/L
> or =18 years: 433-1,532 mcg/L
% REDUCED CoQ10
<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
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, and a phone number to reach a laboratory director in case the referring physician has additional questions.
Cautions Discusses conditions that may cause diagnostic confusion, including improper specimen collection and handling, inappropriate test selection, and interfering substances
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.
Clinical Reference Provides recommendations for further in-depth reading of a clinical nature
1. Littarru GP, Tiano L: Clinical aspects of coenzyme Q10: An update. Nutrition 2010;26:250-254
2. Miles MV, Horn PS, Morrison JA, et al: Plasma coenzyme Q10 reference intervals, but not redox status, are affected by gender and race in self-reported healthy adults. Clin Chim Acta 2003 June;332(1-2):123-132
3. Quinzii CA, Hirano M: Coenzyme Q and mitochondrial disease. Deve Disabil Res Rev 2010 June;16(2):183-188
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
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, et al: HPLC analysis of reduced and oxidized coenzyme Q in human plasma. Clin Chem 2001 February;47:256-265)
Day(s) and Time(s) Test Performed Outlines the days and times the test is performed. This field reflects the day and time the sample must be in the testing laboratory to begin the testing process and includes any specimen preparation and processing time required before the test is performed. Some tests are listed as continuously performed, which means assays are performed several times during the day.
Monday through Friday; 8 a.m.
Analytic Time Defines the amount of time it takes the laboratory to setup and perform the test. This is defined in number of days. The shortest interval of time expressed is "same day/1 day," which means the results may be available the same day that the sample is received in the testing laboratory. One day means results are available 1 day after the sample is received in the laboratory.
3 days (not reported Saturday or Sunday)
Maximum Laboratory Time Defines the maximum time from specimen receipt at Mayo Medical Laboratories until the release of the test result
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 The location of the laboratory that performs the test
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's instructions, or as products that do not undergo full FDA review and approval, and are then labeled as an Analyte Specific Reagent (ASR), Investigation Use Only (IUO) product, or a Research Use Only (RUO) product.
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 Provides guidance in determining the appropriate Current Procedural Terminology (CPT) code(s) information for each test or profile. The listed CPT codes reflect Mayo Medical Laboratories interpretation of CPT coding requirements. It is the responsibility of each laboratory to determine correct CPT codes to use for billing.
LOINC® Code Information Provides guidance in determining the Logical Observation Identifiers Names and Codes (LOINC) values for the result codes returned for this test or profile.
|Result ID||Reporting Name||LOINC Code|
|87853||CoQ10 reduced||In Process|
|30092||CoQ10 % reduced||In Process|