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Test ID PIPA Pipecolic Acid, Serum

Useful For

Differentiating between disorders of peroxisomal biogenesis (eg, Zellweger syndrome) and disorders with loss of a single peroxisomal function


Detecting abnormal elevations of pipecolic acid in serum

Genetics Test Information

In the newborn period, pipecolic acid levels are more likely to be abnormal in urine than in plasma or serum. Abnormal levels of pipecolic acid should be interpreted together with the results of other biochemical markers of peroxisomal disorders, such as plasma C22-C26 very long-chain fatty acids, phytanic acid, pristanic acid, red blood cell plasmalogens, and bile acid intermediates.


Measurement of pipecolic acid is a useful diagnostic tool for differentiating between peroxisomal biogenesis disorders (Zellweger spectrum disorders) and peroxisomal disorders caused by single enzyme deficiencies such as X-linked adrenoleukodystrophy.


Results must be interpreted together with the results of other biochemical markers for peroxisomal disorders.


Both urine and plasma are suitable specimens for the detection of pipecolic acid.

Method Name

Gas Chromatography-Mass Spectrometry (GC-MS)

Reporting Name

Pipecolic Acid, S

Specimen Type


Necessary Information

Patient's age is required.

Specimen Required

Patient Preparation: Fasting 12 hours or more. (Collect specimens from infants and small children just before next feeding)

Collection Container/Tube:

Preferred: Red top

Acceptable: Serum gel

Submission Container/Tube: Plastic vial

Specimen Volume: 1.5 mL

Collection Instructions: Centrifuge and aliquot serum into plastic vial.

Specimen Minimum Volume

1 mL

Specimen Stability Information

Specimen Type Temperature Time Special Container
Serum Frozen (preferred) 94 days
  Refrigerated  14 days

Reference Values

<6 months: ≤6.0 nmol/mL

6 months-<1 year: ≤5.9 nmol/mL

1-17 years: ≤4.3 nmol/mL

≥18 years: ≤7.4 nmol/mL

Day(s) Performed


Performing Laboratory

Mayo Clinic Laboratories in Rochester

CPT Code Information


LOINC Code Information

Test ID Test Order Name Order LOINC Value
PIPA Pipecolic Acid, S 32334-5


Result ID Test Result Name Result LOINC Value
81326 Pipecolic Acid, S 32334-5
29962 Interpretation 59462-2
29964 Reviewed By 18771-6

Test Classification

This test was developed, and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. This test has not been cleared or approved by the US Food and Drug Administration.

Report Available

2 to 9 days

NY State Approved


Testing Algorithm

See Epilepsy: Unexplained Refractory and/or Familial Testing Algorithm in Special Instruction.