Test Catalog

Test ID: PIPU    
Pipecolic Acid, Random, Urine

Useful For Suggests clinical disorders or settings where the test may be helpful

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 urine

Genetics Test Information Provides information that may help with selection of the correct genetic test or proper submission of the test request

Pipecolic acid is not detected by conventional organic acid analysis of urine.


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, RBC 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.

Testing Algorithm Delineates situations when tests are added to the initial order. This includes reflex and additional tests.

Special Instructions Library of PDFs including pertinent information and forms related to the test

Method Name A short description of the method used to perform the test

Gas Chromatography-Mass Spectrometry (GC-MS)

NY State Available Indicates the status of NY State approval and if the test is orderable for NY State clients.


Reporting Name Lists a shorter or abbreviated version of the Published Name for a test

Pipecolic Acid, U