Fig. 4: ERT ameliorated the structural changes and reduced the accumulation of Gb3 in GLA-mutant human iPSC kidney organoids.
From: Human kidney organoids reveal the role of glutathione in Fabry disease
a Representative brightfield image of changes in the morphology of GLA-mutant kidney organoids after ERT. Scale bar = 300 µm. b Quantification of kidney organoid diameters after ERT. c Representative western blot of the expression of GLA after ERT. d Representative image of immunofluorescence staining of Gb3 in GLA-mutant kidney organoids after ERT. Scale bar = 50 µm. e Quantification of the percentage of Gb3-positive area. The values are the mean ± SEM. *p < 0.05; **p < 0.01; ***p < 0.001.
