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Amino Acids Urine Test

Last updated May 11, 2019

Approved by: Maulik P. Purohit MD, MPH

The Amino Acids Urine Test detects the presence of amino acids in urine. It is used to diagnose metabolic disorders involving protein and amino acid processing.

What are other Names for this Test? (Equivalent Terms)

  • AA Urine Test
  • Amino Acids Urine Levels Test

What is the Amino Acids Urine Test? (Background Information)

  • Amino acids are the building blocks of proteins. They are called amino acids because they possess a nitrogen-containing amino group on one end and an acidic group on the other
  • There are 20 natural amino acids. In humans, 9 of these are essential and can be obtained only through the diet. 6 more are semi-essential, meaning they can be made in small quantities, but dietary intake is important. The remaining 5 can be produced in the body and are generally non-essential
  • Amino acids are important for their role in protein formation. Proteins act as enzymes, provide structural support, and are crucial to a wide array of different bodily functions
  • The unique chemical structure of amino acids also helps them protect blood against rapid changes in acidity or basicity. This is known as a buffering effect
  • Amino acids are processed in the liver and kidneys. Various enzymes carry out amino acid degradation and synthesis
  • During amino acid excretion, the nitrogen-containing amino group is removed. It is reactive, however, and therefore must be made into a more stable compound, urea, which is then excreted in the urine. Whole amino acids do not normally appear in urine
  • The problems with the enzymes involved in amino acid processing may result in either excess or deficient amino acid levels in the body. This can cause a variety of metabolic disorders that can lead to abnormal levels of amino acids in urine
  • The Amino Acids Urine Test detects the presence of amino acids in urine. It is used to diagnose metabolic disorders involving protein and amino acid processing. These disorders often stem from genetic defects in metabolic enzymes

What are the Clinical Indications for performing the Amino Acids Urine Test?

Following are the clinical indications for performing the Amino Acids Urine Test:

  • Discolored urine
  • Blood in the urine
  • Seizures
  • Hyperactivity
  • Cognitive impairment
  • Nausea
  • Vomiting
  • Abdominal pain
  • Diarrhea

How is the Specimen Collected for the Amino Acids Urine Test?

Following is the specimen collection process for Amino Acids Urine Test:

Sample required: Urine 

Process of obtaining a urine sample in adults: Urination into a sterile container over a 24-hour period.

Preparation required: No special preparation is needed prior to the test; however, one may be advised to drink 6-8 glasses of water or other fluids prior to the test.

What is the significance of the Amino Acids Urine Test result?

A positive Amino Acids Urine Test finding may indicate:

  • Cystinuria
  • Phenylketonuria
  • Tyrosinosis
  • Ketosis

The laboratory test results are NOT to be interpreted as results of a "stand-alone" test. The test results have to be interpreted after correlating with suitable clinical findings and additional supplemental tests/information. Your healthcare providers will explain the meaning of your tests results, based on the overall clinical scenario.

Additional and Relevant Useful Information:

  • The Amino Acids Urine Test is used as a screen for amino acids present in urine. While it is an important first step in identifying a metabolic disorder, further tests are needed to diagnose the disorder that is present

Certain medications that you may be currently taking may influence the outcome of the test. Hence, it is important to inform your healthcare provider of the complete list of medications (including any herbal supplements) you are currently taking. This will help the healthcare provider interpret your test results more accurately and avoid unnecessary chances of a misdiagnosis.

The following DoveMed website link is a useful resource for additional information:


Please visit our Laboratory Procedures Center for more physician-approved health information:


References and Information Sources used for the Article:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Oct. 31, 2015
Last updated: May 11, 2019