2mecitrateurscnc
A urine test that measures 2-methylcitrate levels to help diagnose and monitor inherited metabolic disorders affecting protein and fat metabolism.
{"2-Methylcitrate is a marker for metabolic disorders like propionic acidemia and methylmalonic acidemia","The test requires a simple urine sample collected in a sterile container","Abnormal results indicate problems with breaking down certain amino acids and fatty acids","Early diagnosis through newborn screening allows for better management of these conditions","Follow-up testing is usually needed to confirm any abnormal findings"}
Key facts at a glance
- LOINC
- 26811-0
- Short code
- 2-MC
- Synonyms
- 2Me-citrate Ur-sCnc
- Category
- chemistry
- Sample
- urine
- Container
- Sterile plastic urine container (60 mL capacity recommended)
- Units
- N/A
- Ref low
- 0
- Ref high
- 2
- Critical range
- — – 20 N/A
- 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
- 83918 (Organic acids, quantitative, urine) or 82128 (Methylmalonic acid, quantitative)
- SNOMED
- 414441006 (Urine 2-methylcitrate measurement)
- Specimen volume
- Minimum 5 mL, preferably 10-15 mL
- Stability
- Room temperature: 24 hours; Refrigerated (2-8°C): 5-7 days; Frozen (-20°C or below): 30 days. Avoid repeated freeze-thaw cycles.
- Hemolysis max
- 0
- Lipemia max
- 0
- Icterus max
- 0
- TAT
- 48–168 hours
- Order
- routine
What is this test?
The 2-methylcitrate urine test measures the amount of 2-methylcitrate in your urine. 2-Methylcitrate is a chemical compound that builds up in the body when certain metabolic pathways do not work properly. This test is part of a group of tests called organic acid analysis, which help doctors identify inherited metabolic disorders.
Why is it done?
This test is primarily used to help diagnose and monitor conditions such as propionic acidemia and methylmalonic acidemia. These are rare genetic disorders where the body cannot properly break down certain amino acids (protein building blocks) and fatty acids. When these disorders are present, 2-methylcitrate and other abnormal chemicals accumulate in the body and can be detected in urine.
How to prepare
No special fasting or preparation is typically required for this test. Your doctor will provide specific instructions. It is important to inform your doctor about any medications or supplements you are taking, as some substances may affect test results. For accurate results, you may be asked to collect a first-morning urine sample.
What happens during the test?
A healthcare provider will give you a sterile container for urine collection. You will provide a urine sample, typically the first morning urine, which is more concentrated and provides better detection of metabolites. The sample is then sent to a laboratory where specialized equipment analyzes the 2-methylcitrate levels.
Understanding your results
Results are reported as moles per volume (mol/L) or as a ratio to creatinine. Normal results vary by age and laboratory. Elevated 2-methylcitrate levels suggest a problem with protein or fat metabolism. Your doctor will interpret your results in context with other tests and your overall health condition.
Reference range
Reference range: 0-2.0 mmol/mol creatinine (age-dependent). Interpretation requires clinical context and correlation with other organic acid levels. Elevated 2-methylcitrate is characteristic of methylmalonic acidemia (MMA) and propionic acidemia (PA).N/A
No special preparation required. Collect random urine specimen or timed collection as ordered. Inform patient that no fasting is necessary. Provide sterile urine container.
Clinical interpretation
How abnormal results are interpreted and what conditions they suggest.
◆Associated findings1
Frequently asked questions
Is this test dangerous?
How long does it take to get results?
Do I need to fast before this test?
What does it mean if my results are abnormal?
Can medications affect my test results?
Sources
- 1
- 2
- 3
- 4
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