Pkd1 regulates immortalized proliferation of renal tubular epithelial cells through p53 induction and JNK activation
J. Clin. Invest. Saori Nishio, et al. 115:910 doi:10.1172/JCI22850 [Go to this article.]

Figure 2
Pkd1^–/–/LZ⁺ mice as an animal model of human ADPKD. (A) Appearance of a P7 Pkd1^–/–/LZ⁺ mouse with an intermediate chimeric rate. The chimeric rate was estimated by coat color. (B) Kidneys of P7 Pkd1^–/–/LZ⁺ mice. Low and Mid (intermediate) indicate the chimeric rate as estimated by coat color: Low, less than 10%; Mid, 10% to approximately 30%. (C) Kidneys of a P60 Pkd1^–/–/LZ⁺ mouse. Black arrowheads indicate hemorrhagic cysts; white arrowhead indicates pancreatic cysts. (D) Cross sections of kidney (Kid), liver, and pancreas (Panc) of a P60 Pkd1^–/–/LZ⁺ mouse. Approximately 90% of the renal parenchyma is occupied by large cysts (PAS staining). Liver and pancreas show numerous cysts (H&E). Original magnification, ×2 (kidney) and ×2.5 (liver and pancreas). (E) Single nephrons of Pkd1^–/–/LZ⁺, Pkd1^–/–, and wild-type mice at E17.5. Multiple “out-pocketing” cysts are present in all segments of the nephron from the Pkd1^–/–/LZ⁺ mouse. Cystic dilation begins at the distal tubule of the nephron of the Pkd1^–/– mouse. Scale bar: 100 μm. (F) Staining of a microdissected tubule with β-gal. A cystic fragment of the Pkd1^–/–/LZ⁺ mouse was composed of Pkd1^+/+ (blue; LZ⁺) and Pkd1^–/– (white; LZ^–) cells. Scale bar: 100 μm. (G) Histochemical analysis of the kidney of a Pkd1^–/–/LZ⁺ mouse at E17.5 with β-gal. The cyst (*) began at tubules involving Pkd1^–/– (LZ^–) and LZ⁺ cells. Some tubules composed of LZ^– cells (black arrowheads) showed no cystic dilatation. Counterstaining: Nuclear Fast Red. Original magnification, ×400.