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C3 Propionylcarnitine

Complement

C3C3 acylcarnitinePropionylcarnitine (C3)

Review status

Currently under review

Pending specialist review and validation.

What it shows

C3 propionylcarnitine is a small molecule formed when propionyl groups are attached to carnitine, a carrier that helps move certain byproducts of protein and fat metabolism. It reflects how your body processes odd‑chain fatty acids and specific amino acids that generate propionyl‑CoA.

This test measures the amount of propionylcarnitine in blood, typically using tandem mass spectrometry. It is commonly included in newborn screening and is also used in children and adults when doctors are evaluating possible metabolic conditions or monitoring known disorders.

Why it matters

Higher C3 propionylcarnitine can be a clue to inherited metabolic conditions such as propionic acidemia or methylmalonic acidemia, which affect how the body breaks down certain nutrients. It can also rise with nutritional vitamin B12 deficiency, especially in breastfed infants of mothers with low B12, and during times of illness or catabolic stress.

Clinicians use this test to screen newborns, to help diagnose people with suggestive symptoms, and to monitor those already diagnosed or receiving treatments like carnitine or vitamin B12. The result is one piece of a bigger picture and is interpreted alongside symptoms, exam findings, and additional laboratory tests.

Understanding your results

If your value is higher than expected for your age, your clinician will consider how you are feeling and may order confirmatory tests. These can include a full acylcarnitine profile, urine organic acids, blood methylmalonic acid, homocysteine, vitamin B12 status, and sometimes genetic testing. In newborns, testing the birth parent’s B12 level can also be important because maternal deficiency can influence an infant’s result.

A result within the typical range is reassuring but does not rule out every metabolic or nutritional problem. A lower value is usually not concerning by itself and can be seen with low carnitine, after successful treatment, or with specimen timing differences. If your result was obtained during acute illness, on parenteral nutrition, or shortly after starting supplements, your clinician may repeat the test once conditions are stable to confirm the finding.

Reference ranges

0.120.45 umol/L
All sexes
0 days – 7 days
0.120.54 umol/L
All sexes
7 days – 1 month
0.260.99 umol/L
All sexes
1 month – 12 months
0.230.77 umol/L
All sexes
12 months – 13 years
0.210.68 umol/L
All sexes
13 years – 19 years
0.130.65 umol/L
All sexes
19 years – 150 years

Reference intervals vary by laboratory, analyzer, methodology, population, and units. The ranges shown here are for education only. Always interpret your results against the reference interval printed on your own lab report.

Factors that could impact C3 Propionylcarnitine

  • Age and newborn transition

    C3 levels naturally shift in the first weeks of life as feeding starts and metabolism matures, so age‑specific interpretation is essential for infants.

  • Illness and fasting

    Fever, vomiting, poor intake, or any catabolic stress can temporarily raise C3 as the body breaks down internal stores for energy.

  • Vitamin B12 status

    Nutritional B12 deficiency in you or, for infants, in the breastfeeding parent can increase C3; correcting B12 often normalizes the result.

  • Medications and nutrition support

    Carnitine supplementation, total parenteral nutrition, and certain specialized diets can alter acylcarnitine patterns, including C3.

  • Sample type and handling

    Differences between dried blood spot and plasma, delays in processing, or hemolysis can affect measured acylcarnitines and should be controlled.

  • Kidney and liver function

    Reduced renal clearance or hepatic dysfunction can change acylcarnitine concentrations and complicate interpretation.

2026

References

  1. McGill University Health Centre. (2019, June 07). C3 Propionyl (Task CD 709457). Laboratory reference ranges.
  2. American College of Medical Genetics and Genomics. (2022). ACT sheet: Elevated C3 (propionylcarnitine) – consider propionic acidemia or methylmalonic acidemia.
  3. Vockley, J., & Ensenauer, R. (2006). Isolated methylmalonic acidemia and propionic acidemia: Review and update. Molecular Genetics and Metabolism, 88(1), 14–27.