School of Science, Monash University Malaysia, 47500, Subang Jaya, Selangor Darul Ehsan, Malaysia.
School of Science, Monash University Malaysia, 47500, Subang Jaya, Selangor Darul Ehsan, Malaysia.
Anal Biochem. 2021 Sep 1;628:114287. doi: 10.1016/j.ab.2021.114287. Epub 2021 Jun 10.
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.
法布里病是由于α-GAL A 活性降低和糖鞘脂（Gb）积累引起的。在这里，我们描述了一种使用荧光标记抗体和粗细胞脂质提取物的微孔板 Gb 测定法。与非病变细胞相比，该测定法能够检测到更高浓度的人 Fabry 细胞中的 Gb。通过免疫荧光染色证实了这一结果，该染色显示 Fabry 细胞系中有大量的 Gb 沉积，证明了该方法的准确性。该测定法可通过定量人生物样品（例如尿液和血液）中的 Gb 沉积来为检测法布里病提供依据。
