Fig. 5

Comparison of Gb-3 deposition and zebra body in kidney organoids. A Representative immunofluorescence staining of Gb3, LTL, and PODXL in WT, CMC-Fb-001, and CMC-Fb-003 kidney organoids. Scale bar = 100 μm. Enlarged image at the podocyte is shown. Scale bar = 20 μm. B Quantification of the percentage of Gb-3 positive area. C Raw and enlarged TEM images of WT, CMC-Fb-001, and CMC-Fb-003 kidney organoids. Red arrows indicate lysosomal Gb-3 depositions. Scale bar = 2 μm for upper figures in C. Scale bar = 0.4 μm for lower figures in B. Gb-3; globotriaocylceramide