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. 2014 Jul 3;26(1):39–47. doi: 10.1681/ASN.2013121312

Figure 1.

Figure 1.

C57BL/6 Pkd1RC/RC mice have milder PKD than the outbred model and respond to dDAVP treatment. (A) Masson's trichrome–stained kidney cross-sections of 3-month-old or 6-month-old inbred and outbred animals. Outbred animals developed higher cyst burdens and larger kidneys compared with inbred mice. Scale bar: 500 μm. (B) Masson's trichrome–stained liver sections of microhamartomas (small, dilated, irregularly shaped bile ducts surrounded by fibrosis). In outbred animals this abnormality was not found before 12 months, but inbred mice developed similar-size microhamartomas as early as 3 months. Scale bar: 100 μm. (C and D) %KW/body wt and %LW/ERbody wt of inbred (green) and outbred (black) mice at 3 and 6 months, depicted as mean diamonds and SDs. (C) The %KW/body wt in inbred mice was less variable among non-littermates but overall was milder and more slowly progressive. Wild-type (WT) C57BL/6 mice also had lower %KW/body wt than WT outbred mice, while body weight remained constant, highlighting a clear background effect in kidney anatomy (%KW/body wt in 10 inbred mice and 4 outbred mice: 3 months, inbred, 1.35%±0.11%, outbred, 1.49%±0.20% [P=0.11]; 6 months, inbred, 1.24%±0.05%, outbred, 1.66±0.24 [P=0.15×10−3) (Supplemental Figure 1A). (D) The %LW/ERbody wt was elevated in inbred compared with outbred mice at 3 months (inbred, 5.39%±0.34%; outbred, 4.80%±0.30%; P=0.21×10−2) and 6 months (inbred, 5.54%±0.40%; outbred, 4.97%±0.48%; P=0.34×10−2). Gray dotted lines represent WT (C57BL/6 or outbred) mice. (E) Gross anatomy of representative kidneys from inbred WT mice, inbred Pkd1RC/RC control mice, and dDAVP-treated mice (6 months of age) highlights increased kidney size after dDAVP treatment. Scale bar: 0.5 cm. (F) Masson's trichrome–stained cross-sections of kidneys with mean±1×SD %KW/body wt. Cross-sections of dDAVP-treated mice showed more severe cystic disease with dilated tubules/ducts (inset, cortex) compared with untreated mice. Scale bar: 500 μm, 250 μm (inset). (G–J) %KW/body wt (G), renal cystic volume (H), renal fibrotic volume (I), and cAMP levels (J) of saline-treated inbred Pkd1RC/RC control mice (C, green) and dDAVP-treated mice (D, purple), depicted as mean diamonds and SDs. Gray dotted lines represent WT values. dDAVP treatment significantly increased %KW/body wt (G), cystic volume (H), and fibrotic volume (I). (J) As predicted, cAMP levels were elevated upon dDAVP treatment. %KW/BW, %KW/body wt. *P<0.05; ** P<0.01; ***P<0.001; ****P<0.0001. Data of outbred animals were obtained from reference 11.