Related Experiment Video
Updated: Jun 2, 2026

Glutamine Flux Imaging Using Genetically Encoded Sensors
Published on: July 31, 2014
Assessment of plasma ammonia and glutamine concentrations in urea cycle disorders
M Serrano1, A Ormazábal, M A Vilaseca
1Neuropediatric Gastroenterology and Clinical Biochemistry Departments, Hospital Sant Joan de Déu, Barcelona, Spain. mserrano@hsjdbcn.org
Objectives:
To analyze the association between ammonia and glutamine used for metabolic control in inherited urea cycle disorders (UCD) in a large series of patients.
Design And Methods:
Paired plasma amino acid-ammonia data from 26 UCD patients were analyzed (n=921).
Results:
Increased plasma glutamine values were consistently observed in UCD patients, despite normal plasma ammonia concentrations, especially for mitochondrial UCD.
Conclusions:
Further therapeutic efforts are probably needed to control increased glutamine values, considering their potentially neurotoxic effect.
Related Concept Videos
Urea Cycle
Serum Studies: Renal Function Tests
Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test
Drug Dosing in Renal Diseases: Measurement of Glomerular Filtration Rate
Inorganic Nitrogen Assimilation
Nephrotic Syndrome II : Assessment and Medical Management

