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 ₃ ). Here, we describe a microplate Gb ₃ assay using fluorophore-tagged antibody and crude cellular lipid extracts. The assay is able to detect higher Gb ₃ concentrations in human Fabry cells compared to non-diseased cells. This result was verified by immunofluorescence staining that revealed large amounts of Gb ₃ deposits in Fabry cell lines, demonstrating the accuracy of this method. This assay may provide the basis for detecting Fabry disease by quantifying Gb ₃ deposits from human biological samples, for example, from urine and blood.
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