Phenotype Image Detail

Image

Caption

Primary cilia are elongated in Pkd1^tm1.1Pcha/Pkd1^tm1.1Pcha (Pkd1^RC/RC) and Pkd1^tm1.1Pcha/Pkd1^tm1Shh (Pkd1^RC/del2) collecting ducts (CDs) but not normal bile ducts. Scanning electron microscopy (SEM) analysis of primary cilia length (A) in the CDs and (B) bile ducts of wild-type (WT), Pkd1^tm1.1Pcha/Pkd1^tm1Shh (Pkd1^RC/del2), and Pkd1^tm1.1Pcha/Pkd1^tm1.1Pcha (Pkd1^RC/RC) mice at the ages indicated (n = 3 animals/group). (A) Representative SEM images of CDs and individual cilia. Scale bars: 2 um (bottom row); 10 um (WT, top row); 15 um (Pkd1^tm1.1Pcha/Pkd1^tm1Shh and Pkd1^tm1.1Pcha/Pkd1^tm1.1Pcha, top row) (left). Graph summarizing > 100 cilia measurements (~40 cilia in nondilated, ~30 in dilated, and ~30 in cystic CDs) in mutant animals and > 50 cilia of nondilated CDs in WT animals (P25 and 12 months). The level of functional Pkd1 inversely correlated with cilia length, indicating a role of PC-1 in cilia maintenance (right). (B) Representative SEM image of WT and Pkd1^tm1.1Pcha/Pkd1^tm1.1Pcha bile duct and one microhamartoma. Bile duct cilia of the microhamartoma were significantly elongated (6.24 +/- 1.57 um, P < 0.0001) (left). Scale bars: 1 um (WT and Pkd1^tm1.1Pcha/Pkd1^tm1.1Pcha, bottom row); 2 um (microhamartoma, bottom row); 50 um (Pkd1^tm1.1Pcha/Pkd1^tm1.1Pcha, top row); 100 um (WT and microhamartoma, top row). Graph summarizing >50 cilia measurements of >5 bile ducts per animal (right). Statistical values were obtained by the Student's t test (**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^tm1.1Pcha/Pkd1^tm1.1Pcha	involves: 129S1/Sv * C57BL/6
Pkd1^tm1Shh/Pkd1^tm1.1Pcha	involves: 129P2/OlaHsd * 129S1/Sv * C57BL/6