Fig. 8. Localization of the ankyrin repeat domain of Inv to the node cilia and its rescue of LR defects of Inv/Inv mice. (A) Schematic representation of the InvC::GFP fusion protein. (B) Phenotype of Inv/Inv mice harboring the InvC::GFP transgene at 2 weeks (a,b) or 4 weeks (c) of age. The transgene rescued the LR defects of Inv/Inv mice as revealed by the normal locations of the stomach and spleen (arrows in a,c) and the normal lobation pattern (L:R, 1:4) of the lungs (b). Although the kidneys appeared normal at 2 weeks (arrowheads in a), they were polycystic at 4 weeks (arrowheads in c). (C) Localization of the InvC::GFP protein in cells of Inv/Inv, InvC::GFP mice. Kidney sections of newborn animals, primary fibroblasts and E8.0 embryos were double immunostained with antibodies to GFP (red, a-c) and antibodies to acetylated tubulin (green, d-f), as indicated. The corresponding DIC images are shown below each pair of fluorescence images (g-i). InvC::GFP was detected in the primary cilia of cultured fibroblasts and in the monocilia of the embryonic node, but not in the 9+0 cilia of the kidney. Arrowheads indicate the positions of cilia.