ArticleJournal of inherited metabolic disease2016
Rapid quantification of underivatized amino acids in plasma by hydrophilic interaction liquid chromatography (HILIC) coupled with tandem mass-spectrometry.
Article in Journal of inherited metabolic disease, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 67 papers.
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67 citing papers in PubMed, 180 citations in OpenAlex.
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- Stress-induced remodeling of yeast amino acid pools occurs within conserved physicochemical constraints.Folia microbiologica · 2026Article
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- Fully Automated Serum LC-MS/MS Platform and Pediatric Reference Intervals for Organic Acids, Amino Acids, and Acylcarnitines in Children (Ages 0-6 Years): Toward Quantitative Diagnosis of Inborn Errors of Metabolism.Diagnostics (Basel, Switzerland) · 2026Article
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- Heterogeneity in clinically diagnosed type 1 diabetes: characterising a unique cohort with maintained C-peptide secretion in Ghana.Diabetologia · 2026Article
- Metabolic adaptations of yeast cells to epigenetic modifiers: a comparative analysis of free amino acid profiles and key metabolites.World journal of microbiology & biotechnology · 2026Article
- CSF protein clearance impairment revealed using stable isotope kinetics in normal pressure hydrocephalus.Brain communications · 2026Article
- Fecal gut microbiota and amino acids as noninvasive diagnostic biomarkers of Pediatric inflammatory bowel disease.Gut microbes · 2025Article
- Comprehensive genotypic, phenotypic, and biochemical characterization of GOT2 deficiency: A progressive neurodevelopmental disorder with epilepsy and abnormal movements.Genetics in medicine : official journal of the American College of Medical Genetics · 2025Article
- S-adenosylmethionine addiction confers sensitivity to methionine restriction inHaematologica · 2025Article
- Metabolic alterations in diabetic patients: aqueous humor profiling for biomarker discovery.Amino acids · 2025Article
- Broad Vitamin BInternational journal of molecular sciences · 2025Article
- From Complexity to Clarity: Expanding Metabolome Coverage With Innovative Analytical Strategies.Journal of separation science · 2025Review
- Isoleucine-to-valine substitutions support cellular physiology during isoleucine deprivation.Nucleic acids research · 2025Article
- Deciphering the immunomodulatory mechanisms ofFrontiers in cell and developmental biology · 2025Article
- Comparative Physiological, Biochemical, and Leaf Proteome Responses of Contrasting Wheat Varieties to Drought Stress.Plants (Basel, Switzerland) · 2024Article
- Comprehensive LC-MS/MS analysis of nitrogen-related plant metabolites.Journal of experimental botany · 2024Article
7 more citing papers are in PubMed but not listed here.
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Authors and funding
8 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundAmino acidopathies are a class of inborn errors of metabolism (IEM) that can be diagnosed by analysis of amino acids (AA) in plasma. Current strategies for AA analysis include cation exchange HPLC with post-column ninhydrin derivatization, GC-MS, and LC-MS/MS-related methods. Major drawbacks of the current methods are time-consuming procedures, derivative problems, problems with retention, and MS-sensitivity. The use of hydrophilic interaction liquid chromatography (HILIC) columns is an ideal separation mode for hydrophilic compounds like AA. Here we report a HILIC-method for analysis of 36 underivatized AA in plasma to detect defects in AA metabolism that overcomes the major drawbacks of other methods.
methodsA rapid, sensitive, and specific method was developed for the analysis of AA in plasma without derivatization using HILIC coupled with tandem mass-spectrometry (Xevo TQ, Waters).
resultsExcellent separation of 36 AA (24 quantitative/12 qualitative) in plasma was achieved on an Acquity BEH Amide column (2.1×100 mm, 1.7 μm) in a single MS run of 18 min. Plasma of patients with a known IEM in AA metabolism was analyzed and all patients were correctly identified.
conclusionThe reported method analyzes 36 AA in plasma within 18 min and provides baseline separation of isomeric AA such as leucine and isoleucine. No separation was obtained for isoleucine and allo-isoleucine. The method is applicable to study defects in AA metabolism in plasma.
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