Galactosylsphingosine does not interfere with the quantitation of plasma glucosylsphingosine levels in Gaucher patients.
Gaucher disease
Glucosylsphingosine
Lyso GL-1
Psychosine
Journal
Clinica chimica acta; international journal of clinical chemistry
ISSN: 1873-3492
Titre abrégé: Clin Chim Acta
Pays: Netherlands
ID NLM: 1302422
Informations de publication
Date de publication:
Jul 2019
Jul 2019
Historique:
received:
20
11
2018
revised:
14
02
2019
accepted:
07
03
2019
pubmed:
13
3
2019
medline:
14
6
2019
entrez:
13
3
2019
Statut:
ppublish
Résumé
It has been shown that the plasma level of glucosylsphingosine (Lyso GL-1) is a useful biomarker for the diagnosis and monitoring of Gaucher disease. Potentially interfering with the quantitation of Lyso GL-1 is its isobaric structural isomer, galactosylsphingosine (psychosine). The contribution of psychosine is generally not accounted for in the determination of Lyso GL-1, due to the difficulty in separating these two isomers. Few methods have been presented in the literature to distinguish the two isomers, and those available tend to be tedious and time-consuming. Here, we developed a LC/MS/MS method able to chromatographically separate Lyso GL-1 and psychosine reproducibly and combine it with a simple, high-throughput sample preparation technique. We also show that the separation of these two isomers in the plasma of Gaucher patients is not necessary for the quantitation of Lyso GL-1 levels, as the relative psychosine level is <3% of Lyso GL-1.
Identifiants
pubmed: 30858093
pii: S0009-8981(19)30114-7
doi: 10.1016/j.cca.2019.03.009
pii:
doi:
Substances chimiques
Psychosine
2238-90-6
sphingosyl beta-glucoside
52050-17-6
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
48-51Informations de copyright
Copyright © 2019 Elsevier B.V. All rights reserved.