Related Experiment Video
Updated: Jun 17, 2026

One-step Metabolomics: Carbohydrates, Organic and Amino Acids Quantified in a Single Procedure
Published on: June 25, 2010
Urine organic acid analysis for inherited metabolic disease using gas chromatography-mass spectrometry
Patricia M Jones1, Michael J Bennett
1Department of Pathology, University of Texas Southwestern Medical Center and Children's Medical Center, Dallas, TX, USA.
Abstract:
Urine organic acid analysis is an essential component of the workup of the patient suspected to have an inborn error of metabolism (IEM). Urine contains several hundred different organic acids, which arise from a multitude of different sources including both normal and abnormal metabolism. They may also arise from drugs and drug metabolism or from xenobiotics and dietary supplements. In addition to the diagnosis of inborn errors of metabolism, the identification of organic acids in a urine sample has a wide range of potential applications, including toxicology and poisonings. The method described below extracts the acidic fraction from urine samples, derivatizes the extracted compounds, and identifies intermediate metabolites by GC-MS. The method utilizes electron impact ionization gas chromatography-mass spectrometry (GC-MS) with total ion collection.
More Related Videos
Related Concept Videos
Urine Studies I: Urinalysis
Mass Spectrometry: Complex Analysis
GC–MS is a powerful hyphenated method commonly used in forensics and environmental...
Inborn Errors of Metabolism
Diagnosing Acidosis and Alkalosis
First, the pH level is assessed to determine whether the blood pH is normal (7.35–7.45), low (acidosis), or high (alkalosis).
Next, the PCO2 and HCO3− values are examined to...

