Figure 5. Conditional mutation of BmprIA gene resulted in urogenital organ defects and reduction in the number of Hh-responsive cells.

BmprIA gene was mutated in Hh-responsive cells by utilizing Gli1^CreERT2/+; BmprIA^flox/flox mice. TM administration dependent hydronephrosis and hydroureter phenotypes (red arrow) were observed at E18.5 (A–C). The Gli1^CreERT2/+; BmprIA^flox/flox embryos displayed the reduced expression of SMA and UPIII in the renal pelvis (D, D’, G, G’), the ureter (E, H) and the bladder trigone region (F, I; yellow boxes). Locations of D’ and G’ were indicated in yellow boxes in D and G. White arrows indicate defective differentiation of cells based on marker expression. Hh-responsive cell contribution assays in BmprIA^flox/flox conditional mutants were performed using the Gli1^CreERT2/+; R26R^LacZ/+ system (J–O). The X-gal stained urinary organs from the control (J–L) and BmprIA^flox/flox; Gli1^CreERT2/+; R26R^LacZ/+ embryos (M–O) following the treatment with TM at E9.5. A reduced number of LacZ positive cells in the renal pelvis (J, M; blue arrows), the ureter (K, N; blue arrows) and the bladder trigone (L, O; blue arrows) were observed in BmprIA^flox/flox; Gli1^CreERT2/+; R26R^LacZ/+ embryos. b: bladder, k: kidney, u: ureter.