Figure 3.

Increased renal AGS3 expression in a mouse model and humans with ADPKD. (A and B) Representative immunoblot analysis is performed in kidneys obtained from Pkd1^v/v hypomorphic mouse (A) and humans with ADPKD kidneys (B). (A) Normal C57Bl/6 and cystic Pkd1^v/v hypomorph kidneys are harvested at postnatal day 14 and analyzed for AGS3 and AGS5/LGN protein expression. The Pkd1^v/v mouse model of ADPKD exhibits a point mutation in the Pkd1 gene, resulting in inefficient cleavage of polycystin 1 (PC1), which is necessary for optimal activity of PC1.²⁸ (B) Normal human kidneys (n = 4) and human ADPKD kidneys (n = 5) are analyzed for the expression of AGS3. (C) Densitometry of the human AGS3 bands are analyzed (P < 0.001). β-actin is used to determine equal loading of the lanes.