Phenotype Image Detail

Image

Caption

Pkd1^tm1.1Pcha/Pkd1^tm1Shh (Pkd1^RC/del2) mice develop in utero/early-onset Autosomal Dominant Polycystic Kidney Disease (ADPKD). (A) Gross images of wild-type (WT) and mutant kidneys from P1 to P180, illustrating early-onset/rapidly progressive disease. After P25, the kidney size decreased, likely due to increased fibrosis. The ruler depicts centimeters. (B) Kaplan-Meier curve of WT and mutant mice. Eighty percent of mutant mice died before P50 (median survival, P28 [arrow]; n = 55/group). (C-E) Graphical representations of percentage of kidney weight per body weight (%KW/BW), blood urea nitrogen (BUN), and cAMP from P1 to P25 of mutant animals. (C) %KW/BW was significantly increased as early as P1 (3%) and rapidly climbed to 25% by P25. (D) BUN levels rose beyond the physiological average as early as P18. (E) cAMP levels were significantly above normal by P1. Values in C-E were obtained from n>/= 5 at each time point for both WT and mutant mice. Statistical values were obtained by the Student's t test (*P < 0.05, **P < 0.01, ***P < 0.001); error bars indicate +/- SD.

Copyright

This image is from Hopp K, J Clin Invest 2012 Nov 1;122(11):4257-73 and is displayed with the permission of the American Society for Clinical Investigation who owns the Copyright. J:193544

Associated Alleles

Symbol	Name
Pkd1^tm1.1Pcha	polycystin 1, transient receptor potential channel interacting; targeted mutation 1.1, Peter Harris
Pkd1^tm1Shh	polycystin 1, transient receptor potential channel interacting; targeted mutation 1, Shigeo Horie

Associated Genotypes

Allelic Composition	Genetic Background
Pkd1^tm1Shh/Pkd1^tm1.1Pcha	involves: 129P2/OlaHsd * 129S1/Sv * C57BL/6