Figure 6.

Immunostaining for the Gb3 Accumulated in Fabry Fibroblasts

Cultured Fabry fibroblasts were stained with anti-Gb3 mAb after addition of the modified NAGA (Modified NAGA), the modified NAGA and 5 mM M6P (Modified NAGA+M6P), agalsidase beta (Agalsidase beta), or agalsidase alfa (Agalsidase alfa). As controls, wild-type cells (Wild-type) and untreated Fabry cells (Fabry) were examined. Each of the enzymes was added to the culture medium to give a concentration of 5 μmol/h/ml, and the cells were cultured for 72 hr. The Gb3 that had accumulated in Fabry fibroblasts was cleaved on administration of the modified NAGA as well as the recombinant GLAs, but not on coadministration of the modified NAGA and M6P. The scale bar represents 50 μm.