An antibody binding-based fluorescent assay for the rapid quantification of globotriaosylceramide levels in human Fabry cells.
Anal Biochem
; 628: 114287, 2021 09 01.
Article
en En
| MEDLINE
| ID: mdl-34119486
ABSTRACT
Fabry disease is caused by reduced α-GAL A activity and accumulation of globotriaosylceramide (Gb3). Here, we describe a microplate Gb3 assay using fluorophore-tagged antibody and crude cellular lipid extracts. The assay is able to detect higher Gb3 concentrations in human Fabry cells compared to non-diseased cells. This result was verified by immunofluorescence staining that revealed large amounts of Gb3 deposits in Fabry cell lines, demonstrating the accuracy of this method. This assay may provide the basis for detecting Fabry disease by quantifying Gb3 deposits from human biological samples, for example, from urine and blood.
Palabras clave
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Trihexosilceramidas
/
Técnica del Anticuerpo Fluorescente
/
Enfermedad de Fabry
Límite:
Humans
Idioma:
En
Revista:
Anal Biochem
Año:
2021
Tipo del documento:
Article
País de afiliación:
Malasia