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Total Acylcarnitine

Metabolic Disorders

Carnitine esters (total)Esterified carnitineTotal AC

Review status

Currently under review

Pending specialist review and validation.

What it shows

Total acylcarnitine measures the amount of carnitine that is carrying fatty acids in your blood. Carnitine helps move fatty acids into mitochondria, where your body turns them into energy. When a fatty acid is attached to carnitine it is called an acylcarnitine; the sum of these is the total acylcarnitine.

This test reflects how your body is handling fat metabolism and the balance between free carnitine and its esterified forms. It is typically performed on a blood sample using mass spectrometry and is often interpreted alongside free carnitine and a detailed acylcarnitine profile.

Why it matters

Clinicians use this test when there is concern for disorders of fatty acid oxidation or carnitine transport, after an abnormal newborn screen, or when symptoms suggest impaired energy metabolism such as low blood sugar, muscle weakness, cardiomyopathy, or liver dysfunction. It can also be useful during critical illness, when the body’s energy use shifts under stress.

Results can help guide diagnosis, treatment decisions such as carnitine supplementation, and monitoring over time. Changes can also reflect kidney or liver function and the effects of certain medications. Interpreting total acylcarnitine together with other tests helps identify whether an inherited condition, nutrition, illness, or drugs are influencing your metabolism.

Understanding your results

Your result is interpreted together with free carnitine, the ratio of acyl to free carnitine, and patterns from a comprehensive acylcarnitine profile. Higher values can be seen when acyl groups accumulate or clearance is reduced, which may occur with inherited fatty acid oxidation defects, significant metabolic stress, or kidney problems. Lower values may reflect limited carnitine availability, reduced intake, or specific transport defects.

One number rarely tells the whole story. Your clinician will consider your symptoms, diet, fasting status, medications, and other labs. Follow‑up may include a full acylcarnitine profile, urine organic acids, plasma amino acids, or genetic testing. If you take carnitine, the timing and dose relative to your blood draw can affect interpretation, so be sure to share these details.

Reference ranges

5.0519.61 umol/L
All sexes
0 days – 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 Total Acylcarnitine

  • Fasting and illness

    Prolonged fasting, fever, or acute illness can shift metabolism toward fat use and increase acylcarnitines, potentially affecting interpretation.

  • Medications and supplements

    Valproate, pivalate‑containing antibiotics, certain antiepileptics, and some antiretrovirals can alter carnitine metabolism. Carnitine supplements raise measured values.

  • Kidney and liver function

    Reduced kidney clearance or liver dysfunction can change acylcarnitine concentrations and patterns independent of genetic disorders.

  • Sample handling

    Hemolysis, delayed processing, or improper storage can degrade or alter analytes, leading to misleading results.

  • Age and physiological state

    Newborns, infants, and pregnant individuals may have different metabolic states, influencing acylcarnitine levels and requiring context‑specific interpretation.

  • Diet and activity

    High‑fat diets, recent heavy exercise, or use of medium‑chain triglycerides can shift acylcarnitine patterns without an underlying genetic disorder.

2026

References

  1. McGill University Health Centre. (2019, May 22). Total Acylcarnitine (Task CD 22048120). Laboratory reference ranges.
  2. American College of Medical Genetics and Genomics. (2020). ACT sheets and confirmatory algorithms: Elevated acylcarnitines. External link
  3. Longo, N., Amat di San Filippo, C., & Pasquali, M. (2016). Primary carnitine deficiency. In M. P. Adam, J. R. Mirzaa, G. M. Pagon, et al. (Eds.), GeneReviews. University of Washington, Seattle. External link
  4. GMDI & Southeast Regional Genetics Network. (2019). Nutrition management guidelines for fatty acid oxidation disorders. External link