Combined analysis of plasma or serum glucosylsphingosine and globotriaosylsphingosine by UPLC-MS/MS.
Clin Chim Acta
; 511: 132-137, 2020 Dec.
Article
en En
| MEDLINE
| ID: mdl-33058838
ABSTRACT
PURPOSE:
To develop a method for the combined analysis of plasma and serum glucosylsphingosine (lyso-Gb1) and globotriaosylsphingosine (lyso-Gb3), biomarkers of Gaucher disease (GD) and Fabry disease (FD), respectively.METHODS:
Internal standards were added to 100 µL of plasma/serum and glycosphingolipids lyso-Gb1, lyso-Gb3, and galactosylsphingosine (GalSph) were extracted with dichloromethane/methanol and analyzed by UPLC-MS/MS. Samples from unaffected controls and patients with GD were first analyzed using a HILIC column to separate lyso-Gb1 from its isomer, GalSph. Samples from patients with FD or GD were analyzed using a C18 column to measure lyso-Gb3 and the hexosylsphingosine (HexSph lyso-Gb1 + GalSph) fraction in a single combined method.RESULTS:
Extraction efficiency was between 73% and 87% and day-to-day variability showed a relative standard error of <7.5%. GalSph was determined to have minimal to no contribution to the HexSph fraction in samples from unaffected controls and patients with GD. Lyso-Gb3 and HexSph measurements by the combined method were in good agreement with established methods, with no bias.CONCLUSIONS:
HexSph and lyso-Gb3 analysis by reversed-phase chromatography UPLC-MS/MS is a cost-effective, time-efficient approach for evaluating these glycosphingolipid biomarkers in patients with a suspected or confirmed diagnosis of GD and FD.Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Psicosina
/
Enfermedad de Fabry
Tipo de estudio:
Diagnostic_studies
/
Guideline
Límite:
Humans
Idioma:
En
Revista:
Clin Chim Acta
Año:
2020
Tipo del documento:
Article
País de afiliación:
Estados Unidos