Figure 6.
Apical sorting of the proton pump is compromised in Ae1+/R607H and Ae1R607H/R607H mice. (A–C) The B1 subunit of the V-type ATPase of acid challenged wild-type (WT) mice almost exclusively localized to the apical domain of A-ICs in the cortex. In Ae1+/R607H and Ae1R607H/R607H mice, however, this shift to the apical domain did not occur, suggesting a secondary targeting defect of the proton pump in Ae1+/R607H and Ae1R607H/R607H mice. Scale bar: 10 µm. (D–I) Immunogold labeling of the (A) subunit of the V-type ATPase. (D–F) Overview of A-ICs from WT, Ae1+/R607H, and Ae1R607H/R607H mice. (G–I) Higher magnification view of the apical cell pole. Scale bar: 2 µm. (J) Renal cortex immunoblot shows reduced B1 subunit expression in both heterozygous and homozygous Ae1 R607H mice. Data are shown as mean±SEM. *P<0.05; ****P<0.0001 (one-way ANOVA and Tukey multiple comparison).