Fig. 5: Treatment with 4-PBA restores α5(IV) expression in mild AS patient iPSC-derived kidney organoids.
From: iPSC-derived type IV collagen α5-expressing kidney organoids model Alport syndrome
a Experimental protocol. b Immunostaining images of glomerulus-like structures in mild AS patient iPSC-derived kidney organoids treated with medium, 4-PBA, TMAO, and mannitol for WT1, α5(IV), and α2(IV). c Immunostaining images of glomerulus-like structures in severe AS patient iPSC-derived kidney organoids treated with medium and 4-PBA for WT1, α5(IV), and α2(IV). Arrows and arrowheads indicate α5(IV)- and α2(IV)-expressing GBM-like structures, respectively. Scale bars, 50 µm.
