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C0/AC Total Ratio (Free Carnitine to Total Acylcarnitines)

Immunology & Autoimmune

C0/ACCarnitine ratioFree Carnitine to Total Acylcarnitines Ratio

Review status

Currently under review

Pending specialist review and validation.

What it shows

This test measures the ratio of free carnitine (often called C0) to the total amount of acylcarnitines in your blood. Carnitine helps move fatty acids into cells so they can be used for energy, while acylcarnitines form when fatty acids are being processed. Looking at the balance between these two gives a snapshot of how your body is handling fat metabolism.

It is typically performed as part of a metabolic evaluation or acylcarnitine profile. Your clinician may use it to understand your carnitine status, to assess how well your body is converting fats to energy, or to monitor carnitine supplementation.

Why it matters

The C0/AC ratio can help identify patterns seen in carnitine deficiency and in disorders of fatty acid oxidation. It is often ordered after an abnormal newborn screen, or when there are symptoms such as poor feeding in infants, low energy, muscle weakness, episodes of low blood sugar, heart enlargement, or unexplained liver issues. In adults, it may be used when there is concern for secondary carnitine depletion due to illness or medications.

This ratio can also help monitor response to L-carnitine therapy and may flag drug effects that lower carnitine stores. Your provider uses it alongside other tests and your history to decide whether more metabolic or genetic testing is needed.

Understanding your results

Your healthcare provider will interpret the ratio in the context of your age, symptoms, recent diet, and any supplements or medicines. A higher ratio can be seen when free carnitine is relatively abundant or acylcarnitines are reduced, including during carnitine supplementation or recovery from illness. A lower ratio can occur when carnitine is depleted or when acylcarnitines are increased by metabolic stress or certain inherited conditions.

If your result is unexpected, your clinician may repeat testing when you are clinically stable, review your fasting status, and order related studies such as a full acylcarnitine profile, free and total carnitine, urine organic acids, ketones, or genetic testing. Results are best interpreted with a specialist familiar with metabolic testing, and changes to diet or medication should be made only after medical advice.

Reference ranges

1.985.42 nan
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 C0/AC Total Ratio (Free Carnitine to Total Acylcarnitines)

  • Fasting and sample timing

    Recent meals, especially high-fat intake, and the timing of the blood draw can shift acylcarnitine levels and affect the ratio. Your provider may prefer a fasting sample for a clearer metabolic picture.

  • Supplements and medications

    L-carnitine supplements can raise free carnitine and increase the ratio, while drugs like valproic acid or pivalate-containing antibiotics can reduce carnitine stores and lower the ratio.

  • Acute illness and metabolic stress

    Fever, infection, prolonged fasting, or heavy exercise can increase acylcarnitines and temporarily alter the ratio. Testing during recovery may yield different results.

  • Age, pregnancy, and prematurity

    Normal carnitine handling varies with age, and values can shift during pregnancy or in premature infants. Interpretation often uses age-appropriate context.

  • Kidney and liver function

    Reduced kidney or liver function can change carnitine balance by altering production, metabolism, or excretion, which may influence the ratio.

  • Specimen handling and matrix

    Delays in processing, improper storage, or differences between serum, plasma, and dried blood spot testing can affect results. Laboratories follow strict procedures to ensure accuracy.

2026

References

  1. McGill University Health Centre. (2018, December 04). C0/AC Totale (Task CD 22048126). Laboratory reference ranges.
  2. Clinical and Laboratory Standards Institute. (2013). Newborn screening by tandem mass spectrometry; Approved guideline (CLSI document NBS04-A).
  3. American College of Medical Genetics and Genomics. (2012). Laboratory standards and guidelines for biochemical genetic testing and newborn screening of acylcarnitines by tandem mass spectrometry.