Blood group B glycosphingolipids (B-GSLs) are substrates of the lysosomal alpha-galactosidase A (AGAL). Similar to its major substrate–globotriaosylceramide (Gb3Cer)–B-GSLs are not degraded and accumulate in the cells of patients affected by an inherited defect of AGAL activity (Fabry disease—FD).

中文翻译：

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带有B型血型的法布里病患者的外分泌胰腺中具有末端α-半乳糖基部分的糖缀合物的特异性储存

血型B糖鞘脂（B-GSL）是溶酶体α-半乳糖苷酶A（AGAL）的底物。与其主要底物-globotriaosylceramide（Gb3Cer）相似，B-GSL不会降解并在受AGAL遗传性遗传缺陷（法布里病-FD）影响的患者细胞中积聚。