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HelpOrder Code LABGOR, MMPP Mitochondrial Metabolites, Plasma
Additional Codes
Mayo Test Code: MMPP
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 |
Reject Due To
| Gross hemolysis | Reject |
| Gross lipemia | OK |
| Gross icterus | OK |
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
Method Description
Plasma specimen is spiked with a mixture of labeled internal standards following oximation of keto acids. The samples are acidified and extracted. After evaporation, the dry residue is silylated and analyzed by capillary gas chromatography/mass spectrometry using selected ion monitoring with positive electron impact ionization and stable isotope dilution.(Unpublished Mayo method)
Day(s) Performed
Wednesday
Performing Laboratory
Mayo Clinic Laboratories in Rochester
CPT Code Information
82542