Fig. 2

Comparison of α-GalA expression, α-GalA activity, and amount of Gb-3 deposition in hiPSC cells by immunofluorescent study and LC–MS/MS. A Representative Western blot and quantification graphs of the expression of α-GalA in hiPSC. B Quantification of α-GalA activity analysis. C Immunofluorescence staining with an antibody against Gb-3 in WT, CMC-Fb-001, and CMC-Fb-003. D Quantification of Gb-3 level in hiPSCs. Mean values from three independent experiments are shown with standard deviation error bars. α-GalA, α-galactosidase A; Gb-3; globotriaocylceramide, WT, wild-type; hiPSC, human induced pluripotent stem cells