(A) Whole-mount immunofluorescence analysis of 3D kidney organoids (Day
24) derived from ARPKD iPSCs and corrected-ARPKD iPSCs. Scale bars, 500
μm.
(B) Representative bright-field images of 3D kidney organoids derived
from H9 embryonic stem cells (ESCs), corrected-ARPKD iPSCs, and ARPKD iPSCs, in
the absence or presence of forskolin (FSK)/8-br-cAMP for 3 days (Day 28 to 31).
Scale bars, 500 μm.
(C) Left panels: time course whole-mount immunofluorescence analysis of
ARPKD kidney organoids treated with 10 μM FSK. Right most panel:
representative H&E staining image of ARPKD kidney organoid treated with 10
μM FSK for 7 days (Day 28 to 35). Scale bars, 500 μm.
(D) Immunofluorescence analysis of ARPKD kidney organoids in the
absence or presence of 10 μM FSK for 3 days (Day 28 to 31) followed by
in vitro dextran uptake assays. White asterisk indicates
cyst. Scale bars, 50 μm.