An antibody binding-based fluorescent assay for the rapid quantification of globotriaosylceramide levels in human Fabry cells.

Fabry disease is caused by reduced α-GAL A activity and accumulation of globotriaosylceramide (Gb). Here, we describe a microplate Gb assay using fluorophore-tagged antibody and crude cellular lipid extracts. The assay is able to detect higher Gb concentrations in human Fabry cells compared to non-diseased cells. This result was verified by immunofluorescence staining that revealed large amounts of Gb deposits in Fabry cell lines, demonstrating the accuracy of this method. This assay may provide the basis for detecting Fabry disease by quantifying Gb deposits from human biological samples, for example, from urine and blood.