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CSF Proline

Body Fluids

Cerebrospinal fluid prolineCSF Pro

Review status

Currently under review

Pending specialist review and validation.

What it shows

CSF Proline measures the concentration of the amino acid proline in cerebrospinal fluid, the clear liquid that cushions your brain and spinal cord. Proline is a building block of proteins and is involved in collagen formation and cellular metabolism. This test is typically performed as part of a focused amino acid evaluation when neurological or metabolic conditions are being considered.

Proline levels in cerebrospinal fluid reflect the balance between levels in the blood, transport across the blood brain barrier, and local metabolism within the central nervous system. The sample is obtained by lumbar puncture and analyzed in a specialized laboratory.

Why it matters

Your clinician may order this test when there are neurologic symptoms such as seizures, developmental concerns, or unexplained regression, especially when an inborn error of metabolism is possible. Marked changes in proline can be seen in genetic conditions that affect proline breakdown or utilization, and the result can help direct additional testing.

Findings are usually interpreted together with plasma and urine amino acids, clinical history, and imaging, since patterns across body fluids provide the clearest picture. Understanding whether the central nervous system is exposed to abnormal proline levels can guide nutrition advice, medication choices, genetic counseling, and treatment monitoring.

Understanding your results

Results are interpreted in the context of your age, symptoms, and other laboratory data. A small deviation near the expected range may be related to diet, recent illness, or a sample issue. Larger or persistent changes raise the possibility of a metabolic disorder and often lead to confirmation in blood and urine, followed by enzyme or genetic testing if indicated.

If your result is higher than expected, your clinician may review medications and nutrition, check kidney and liver function, and consider repeating the test if there were collection or processing concerns such as blood contamination. If your result is lower than expected, your care team will usually focus on the overall clinical picture, since isolated low values are uncommon and rarely require urgent action.

Reference ranges

06 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 CSF Proline

  • Collection quality

    A traumatic lumbar puncture with blood contamination can raise amino acid readings in CSF. Proper technique and discarding the first drops help reduce contamination risk.

  • Handling and storage

    Delayed processing, bacterial growth, or lack of freezing can alter amino acid stability. Rapid transport on ice and prompt freezing support reliable results.

  • Diet and nutrition

    Recent high protein intake, collagen or proline supplements, or parenteral nutrition can influence amino acid levels and may affect CSF values indirectly.

  • Medications

    Some antiepileptics, corticosteroids, and other drugs can affect amino acid metabolism. Provide a complete medication and supplement list to your clinician.

  • Inflammation or infection

    Central nervous system inflammation, meningitis, or high CSF protein can change measured amino acids, complicating interpretation.

  • Renal and liver function

    Kidney or liver dysfunction can shift systemic amino acid levels, which may influence transport into CSF and the measured concentration.

2026

References

  1. McGill University Health Centre. (2006, September 13). CSF Proline (Task CD 693399). Laboratory reference ranges.
  2. Blau, N., Duran, M., Gibson, K. M., & Dionisi-Vici, C. (2014). Physician’s guide to the diagnosis, treatment, and follow-up of inherited metabolic diseases. Springer.
  3. Phang, J. M., & Valle, D. (2018). Hyperprolinemia type I and type II. In M. P. Adam, et al. (Eds.), GeneReviews. University of Washington, Seattle.