Figure 3 | Scientific Reports

Figure 3

From: NEDD4-family E3 ligase dysfunction due to PKHD1/Pkhd1 defects suggests a mechanistic model for ARPKD pathobiology

Figure 3

Smurf2 is mis-localized in Pkhd1 mutants. (a) Sub-cellular localization of Smurf2 (red), Lamp2 (green) and DAPI (blue) in primary cultures of collecting duct cells of wild type (WT) and Pkhd1 del3-4/del3-4 (M) mouse kidneys. Nuclei are stained with DAPI. Scale bars, 10 μm. (b) Wild type (WT) and Pkhd1 del3-4/del3-4 mouse kidneys stained for Smurf2 (red), Lamp2 (green), and DAPI (blue). Scale bars, 10μm. (c) Kidney specimens from human control (control) and two different ARPKD donors (ARPKD1, ARPKD2) stained for DBA (grey), LAMP1 (green), SMURF2 (red) and DAPI (blue). Scale bars, 20 μm.

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