Phenotype Image Detail

Image

Caption

Histology of homozygous Pkd1^tm1.1Pcha (Pkd1^RC/RC) animals mimics typical Autosomal Dominant Polycystic Kidney Disease (ADPKD). (A) Representative Masson Trichrome histology sections of Pkd1^tm1.1Pcha/Pkd1^tm1.1Pcha (Pkd1^RC/RC) and wild-type (WT) mouse kidneys at 1 to 12 months show that cysts developed progressively with age. At later time points, an increased level of fibrosis was noted (12 months). Scale bars: 1 mm (kidney cross-section, WT, and Pkd1^RC/RC); 250 um (Pkd1^RC/RC magnified region [images in second column]); 200 um (WT magnified region [insets]). (B) Masson Trichrome histology sections of 12-month-old Pkd1^RC/RC and WT livers (no significant difference between percentage of liver weight [LW] per body weight [%LW/BW] of WT animals and homozygotes was noted). Four out of five homozygous animals showed ductal plate malformations, including microhamartomas of varying severity. These cystic lesions are progenitors of the liver cysts often found in patients with ADPKD. Scale bars: 100 um.

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

Associated Genotypes

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