OROT - Overview: Orotic Acid, Urine

Test Catalog

Test Name

Test ID: OROT    
Orotic Acid, Urine

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

Evaluation of the differential diagnosis of hyperammonemia and hereditary orotic aciduria


When orotic acid is measured after a protein load or administration of allopurinol, excretion of orotic acid is a very sensitive indicator of ornithine transcarbamylase (OTC) activity. An allopurinol challenge may be helpful in determining whether a female patient may be a carrier of an OTC mutation if molecular genetic testing was not informative.

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


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


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

Orotic Acid, U

Aliases Lists additional common names for a test, as an aid in searching

Cit I
Citrullinemia I
Urea Cycle Disorder (UCD)
OTC (Ornithine Transcarbamylase)
ASA (Aspirin)
Argininosuccinic Aciduria
Hereditary Orotic Aciduria
Hyperornithinemia, Hyperammonemia, Homocitrullinuria Syndrome
Lysinuric Protein Intolerance
Ornithine Transcarbamylase Deficiency
Uridine Monophosphate Synthase Deficiency