FHH Shroom3 is defective and contributes to glomerular dysfunction. (A) Schematic representation of the rat Shroom3 protein is shown. Vertical lines represent the amino acid variants found in FHH Shroom3. Variants predicted to be damaging by PolyPhen-2 are shown in red. (B) Co-injection of shroom3 + tp53 morpholino (MO) with full-length BN, but not FHH, Shroom3 mRNA rescued the edema phenotype. ([***] P < 0.001 vs. MO and MO + FHHmRNA) and (C) cell death induced by shroom3 + tp53 MO ([*] P < 0.05 vs. uninjected control). (D) Representative fluorescence images of individual dorsal aorta at 1, 24, and 48 h following 70-kDa dextran injection are shown. (E) Co-injection with BN but not FHH Shroom3 mRNA rescued the dextran leakage induced by knockdown of endogenous shroom3 in zebrafish. (n = 35, 26, 23, and 22, respectively. [*] P < 0.05 vs. uninjected and MO + BNmRNA.)
