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HelpTest ID MMPP Mitochondrial Metabolites, Plasma
Ordering Guidance
This test is not the recommended initial screening test for evaluating patients with suspected mitochondrial disorders, organic acidurias, and ketone body disorders. For these purposes, the preferred tests for first-tier assessment are OAU / Organic Acids Screen, Random, Urine; AAQP / Amino Acids, Quantitative, Plasma; and ACRN / Acylcarnitines, Quantitative, Plasma.
Analytes from LAPYP / Lactate Pyruvate Panel, Plasma are included in this test. If ordered together, LAPYP may be canceled.
Specimen Required
Collection Container/Tube:
Preferred: Green top (Sodium heparin)
Acceptable: Green top (Lithium heparin)
Submission Container/Tube: Plastic vial
Specimen Volume: 0.5 mL
Collection Instructions: Centrifuge and aliquot plasma into a plastic vial.
Forms
If not ordering electronically, complete, print, and send a Biochemical Genetics Test Request (T798) with the specimen.
Useful For
Monitoring patients with mitochondrial disorders, organic acidurias, and ketone body disorders
Method Name
Gas Chromatography Mass Spectrometry (GC-MS)
Reporting Name
Mitochondrial Metabolites, PSpecimen Type
PlasmaSpecimen Minimum Volume
0.1 mL
Specimen Stability Information
| Specimen Type | Temperature | Time | Special Container |
|---|---|---|---|
| Plasma | Frozen | 7 days |
Reference Values
LACTIC ACID
≤ 4000.0 nmol/mL
2-HYDROXYBUTYRIC ACID
≤ 124.0 nmol/mL
3-HYDROXYBUTYRIC ACID
≤ 700.0 nmol/mL
PYRUVIC ACID
≤ 350.0 nmol/mL
cis-ACONITIC ACID
≤ 9.0 nmol/mL
CITRIC ACID
≤ 250.0 nmol/mL
3-HYDROXYPROPIONIC ACID
≤ 12.4 nmol/mL
3-HYDROXY-2-METHYLBUTYRIC ACID
≤ 2.5 nmol/mL
3-HYDROXYISOVALERIC ACID
≤ 15.4 nmol/mL
SUCCINIC ACID
≤ 10.0 nmol/mL
FUMARIC ACID
≤ 5.0 nmol/mL
3-METHYLGLUTACONIC ACID
≤ 1.6 nmol/mL
MALIC ACID
≤ 20.0 nmol/mL
2-KETOBUTYRIC ACID
≤ 16.0 nmol/mL
2-KETOISOVALERIC ACID
≤ 35.0 nmol/mL
ACETOACETIC ACID
≤ 350.0 nmol/mL
3-METHYL-2-KETOVALERIC ACID
≤ 70.0 nmol/mL
2-KETOISOCAPROIC ACID
≤ 70.0 nmol/mL
2-METHYLCITRIC ACID
≤ 1.0 nmol/mL
2-KETOGLUTARIC ACID
≤ 40.0 nmol/mL
Day(s) Performed
Wednesday
Report Available
3 to 9 daysPerforming Laboratory
Mayo Clinic Laboratories in Rochester
Test Classification
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
82542
LOINC Code Information
| Test ID | Test Order Name | Order LOINC Value |
|---|---|---|
| MMPP | Mitochondrial Metabolites, P | 101455-4 |
| Result ID | Test Result Name | Result LOINC Value |
|---|---|---|
| 616819 | Interpretation | 59462-2 |
| 616798 | Lactic acid | 2524-7 |
| 616799 | 2-Hydroxybutyric acid | 69843-1 |
| 616800 | 3-Hydroxybutyric acid | 6873-4 |
| 616801 | Pyruvic acid | 32338-6 |
| 616802 | cis-Aconitic acid | 75083-6 |
| 616803 | Citric acid | 15038-3 |
| 616804 | 3-Hydroxypropionic acid | 47536-8 |
| 616805 | 3-Hydroxy-2-methylbutyric acid | 69789-6 |
| 616806 | 3-Hydroxyisovaleric acid | 72450-0 |
| 616807 | Succinic acid | 35871-3 |
| 616808 | Fumaric acid | 75081-0 |
| 616809 | 3-Methylglutaconic acid | 33273-4 |
| 616810 | Malic acid | 75068-7 |
| 616811 | 2-Ketobutyric acid | In Process |
| 616812 | 2-Ketoisovaleric acid | 35868-9 |
| 616813 | Acetoacetic acid | 35867-1 |
| 616814 | 3-Methyl-2-ketovaleric acid | 35869-7 |
| 616815 | 2-Ketoisocaproic acid | 35870-5 |
| 616816 | 2-Methylcitric acid | 26904-3 |
| 616817 | 2-Ketoglutaric acid | 69803-5 |
| 616818 | Reviewed by | 18771-6 |