PIPU - Overview: Pipecolic Acid, Urine

Test Catalog

Test Name

Test ID: PIPU    
Pipecolic Acid, 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 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 (X-ALD).


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.

Special Instructions and Forms Describes specimen collection and preparation information, test algorithms, and other information pertinent to test. Also includes pertinent information and consent forms to be used when requesting a particular test

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

Gas Chromatography-Mass Spectrometry (GC-MS)

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


Reporting Name A shorter/abbreviated version of the Published Name for a test; an abbreviated test name

Pipecolic Acid, U