2mecitrateurmcnc
A urine test that measures 2-methylcitrate levels to help diagnose and monitor certain metabolic disorders.
{"2-Methylcitrate is a substance that can build up in urine when the body cannot properly process certain amino acids","This test helps doctors diagnose conditions like propionic acidemia and methylmalonic acidemia","It is typically ordered when a baby shows symptoms of a metabolic disorder or as part of newborn screening follow-up","The test involves collecting a urine sample, usually at home","Results help doctors plan appropriate treatment and dietary management"}
Key facts at a glance
- LOINC
- 12539-3
- Short code
- 2-MC Urine
- Synonyms
- 2Me-citrate Ur-mCnc
- Category
- chemistry
- Sample
- urine
- Container
- Sterile, labeled urine container (plastic or glass)
- Units
- N/A
- Ref low
- 0
- Ref high
- 10
- Fasting
- false
- Population
- adult
- Sex
- all
- Age range
- ≥ 0 years
- Method
- Gas Chromatography-Mass Spectrometry (GC-MS) or Liquid Chromatography-Tandem Mass Spectrometry (LC-MS/MS)
- CPT
- 80378 (Organic acids quantitative, urine)
- SNOMED
- 122598009 (2-Methylcitrate measurement)
- Specimen volume
- Minimum 1 mL, preferably 2-5 mL of urine
- Stability
- Room temperature: 24-48 hours; Refrigerated (2-8°C): 5-7 days; Frozen (-20°C or below): Several weeks to months. Protect from light.
- Hemolysis max
- 0
- Lipemia max
- 0
- Icterus max
- 0
- TAT
- 48–168 hours
- Order
- routine
What is this test?
The urine 2-methylcitrate test measures the amount of 2-methylcitrate (also called 2-methylcitric acid) in your urine. 2-Methylcitrate is a normal metabolic byproduct that is usually present in very small amounts. However, when the body cannot properly break down certain proteins and amino acids, 2-methylcitrate can accumulate and appear in higher than normal amounts in the urine.
Why is this test ordered?
This test is primarily ordered to help diagnose and monitor inherited metabolic disorders, specifically propionic acidemia and methylmalonic acidemia. These are rare genetic conditions where the body lacks certain enzymes needed to process specific amino acids (isoleucine, valine, methionine, and threonine). When these conditions are suspected—often due to symptoms like vomiting, poor feeding, developmental delays, or as follow-up to abnormal newborn screening results—this test can provide important diagnostic information.
How is the sample collected?
A urine sample is collected in a clean container. For accurate results, it is best to collect the first morning urine, as this provides the most concentrated sample. The collection is usually done at home, and the sample should be delivered to the laboratory as soon as possible, typically within a few hours. Sometimes you may be asked to collect all urine produced over a 24-hour period for more accurate quantification.
What do the results mean?
Normal urine typically has very low or undetectable levels of 2-methylcitrate. Elevated levels suggest that the body is not properly metabolizing certain amino acids, which may indicate propionic acidemia, methylmalonic acidemia, or other related metabolic disorders. Your doctor will interpret the results along with other tests (such as plasma amino acids and acylcarnitine profile) to make an accurate diagnosis.
What happens after the test?
If the test shows abnormal levels, your doctor will likely order additional tests to confirm the diagnosis. Treatment for these metabolic disorders typically includes a special low-protein diet, medication, and close monitoring. Early diagnosis and treatment are important for better outcomes, which is why newborn screening programs include this type of testing.
Reference range
Reference range varies significantly by laboratory and methodology. Typically reported as mmol/mol creatinine. Consult laboratory-specific reference intervals. Generally, normal excretion is <10 mmol/mol creatinine in newborns and lower in older children and adults.N/A
No special preparation required. Collect random urine sample, preferably first morning void. Avoid contamination with fecal matter. Inform laboratory of any medications or supplements that may affect organic acid metabolism.
Clinical interpretation
How abnormal results are interpreted and what conditions they suggest.
◆Associated findings1
Frequently asked questions
Is this test painful?
Do I need to fast before the test?
Can this test be done on newborns?
How long does it take to get results?
Is treatment available if the test is abnormal?
Sources
- 1
- 2
- 3
Click any link to access the full source. PubMed Central (PMC) links provide free full-text access.
This information is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment.
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