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2mecitrateserplmcnc

Published: August 9, 2026Updated: August 16, 2026

A blood test that measures 2-methylcitrate levels to help diagnose and monitor inherited metabolic disorders affecting protein and fat metabolism.

Quick summary

{"2-Methylcitrate is a marker for propionic acidemia and methylmalonic acidemia","The test requires a simple blood draw from a vein","Abnormal results may indicate a metabolic disorder requiring treatment","Early diagnosis and monitoring are crucial for managing these conditions","This test is often part of newborn screening or follow-up testing"}

Medically reviewed by WellAlly Editorial TeamLast reviewed August 16, 2026Editorial policy

Key facts at a glance

LOINC
12540-1
Short code
2-MC
Synonyms
2Me-citrate SerPl-mCnc
Category
chemistry
Sample
serum
Container
Serum separator tube (SST) or plain red top tube
Units
N/A
Ref low
0
Ref high
0.5
Fasting
false
Population
adult
Sex
all
Age range
≥ 0 years
Method
Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)
CPT
82017 (Organic acids, quantitative, each)
SNOMED
122269004
Specimen volume
1.0-2.0 mL of serum minimum
Stability
Serum should be separated within 2 hours of collection. Store at 2-8°C if analyzing within 48 hours; for longer storage, freeze at -20°C or -70°C. Avoid repeated freeze-thaw cycles.
Hemolysis max
500
Lipemia max
1000
Icterus max
500
TAT
72–168 hours
Order
routine

What is this test?

The 2-methylcitrate test measures the amount of 2-methylcitrate in your blood (serum or plasma). 2-Methylcitrate is a chemical byproduct (metabolite) that builds up in the blood when the body cannot properly break down certain amino acids and fatty acids. This test helps doctors identify inherited metabolic disorders, particularly propionic acidemia (PA) and methylmalonic acidemia (MMA).

Why is this test done?

This test is performed to: 1) Screen newborns for certain metabolic disorders as part of routine newborn screening programs; 2) Diagnose suspected metabolic disorders when symptoms such as poor feeding, vomiting, lethargy, or developmental delays are present; 3) Monitor patients already diagnosed with propionic acidemia or methylmalonic acidemia to evaluate how well treatment is working; and 4) Help differentiate between different types of metabolic disorders.

How is the test performed?

A healthcare professional will draw a small blood sample from a vein in your arm using a needle. The blood is collected into a special tube and sent to a laboratory for analysis. The test requires fasting (not eating) for several hours before the blood draw, as instructed by your doctor. For newborns, blood may be collected from a heel stick.

What do the results mean?

Normal 2-methylcitrate levels vary by age and laboratory. Elevated levels typically suggest propionic acidemia or methylmalonic acidemia. Your doctor will interpret your results alongside other test results and your clinical symptoms. If you have been diagnosed with a metabolic disorder, your doctor will use the results to adjust your treatment plan, which may include dietary changes, medication, or other therapies.

Are there any risks?

The risks of this test are minimal and include slight pain or bruising at the needle site, dizziness, or very rarely, infection. These risks are the same as with any standard blood draw. The benefits of getting an accurate diagnosis or monitoring a serious condition far outweigh these minor risks.

Reference range

Reference range varies by laboratory and method. Typically: <0.5 μmol/L (may vary)N/A

Specimen: serum

No special preparation required. Inform laboratory of any medications or supplements. Collect as part of metabolic disorder workup when inherited metabolic disease is suspected.

Clinical interpretation

How abnormal results are interpreted and what conditions they suggest.

◆Associated findings1

Frequently asked questions

Do I need to prepare for this test?
Your doctor may ask you or your child to fast (not eat) for several hours before the blood draw. Follow any specific instructions provided by your healthcare provider. Inform your doctor about any medications or supplements you are taking, as some may affect test results.
How long does it take to get results?
Results are typically available within a few days to a week, depending on the laboratory. Your healthcare provider will contact you with the results or discuss them at your follow-up appointment.
What happens if my results are abnormal?
If your 2-methylcitrate levels are abnormal, your doctor may recommend additional tests to confirm a diagnosis, such as genetic testing or other metabolic panels. If a metabolic disorder is confirmed, you will be referred to a specialist (metabolic geneticist) for ongoing management and treatment.
Can this test diagnose other conditions?
While 2-methylcitrate is primarily a marker for propionic acidemia and methylmalonic acidemia, abnormal levels may sometimes be seen in other metabolic conditions. Your doctor will consider the full clinical picture and order additional tests as needed.
Is this test only for children?
No, this test can be performed at any age. While it is commonly part of newborn screening, it is also used to diagnose metabolic disorders in older children and adults who may have milder forms that were not detected at birth.
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This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.

Medical Disclaimer

This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.

If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.

Medically reviewed by WellAlly Editorial Team Last reviewed August 16, 2026. · Read our editorial policy