Figure 1. Desmosomes are mispolarized in ADPKD cells in vitro and in situ.
a. Confocal images of normal and ADPKD cells in culture labeled for desmosomal components (green). Upper panels: xy view to show desmosomal appearance at lateral membrane. Lower panels: xz view to show desmosomal distribution between apical and lateral cell domains. Desmoplakin labeling (two left panels) is seen only at the lateral domain of normal kidney cells (NK) but apical and lateral in ADPKD cells (PKD). Plakoglobin (two right panels) in normal (NK) and ADPKD cells (PKD) reveals same phenomenon. Nuclear staining, used as structural reference is seen in blue. Bars: 20 μm (except first panel: 5μm)
b. Western blot of streptavidin-precipitated, biotinylated proteins, blotted with anti-desmoglein antibody. Only basolateral protein (bl) was recovered in normal kidney cells (N), while basolateral (bl) and apical (ap) desmoglein is seen in ADPKD cells (P).
C. Confocal image of cryosections of normal (two left panels) and polycystic (right panel) tissue. Labeling against desmoplakin shows apical staining of the cyst lining epithelia in polycystic kidney tissue (arrowheads) while only lateral signal can be observed in normal tubules (arrows). Bars: 30 μm