Evidence for a “Pathogenic Triumvirate” in Congenital Hepatic Fibrosis in Autosomal Recessive Polycystic Kidney Disease

Figure 2

Cyst wall epithelial cell proliferation, fibrosis, and inflammation in PCK rats. (a) Hyperproliferation of cystic epithelia in polycystic kidney (PCK) rats. Hepatic PCNA content was assessed in Sprague-Dawley (SD) rats and PCK rats by immunohistochemistry. Images were taken at 400x magnification. Arrows indicate PCNA-positive cyst wall epithelial cells (CWECs) and asterisks indicate hepatic cysts. (b) Pericystic fibrosis in PCK rats. Extracellular matrix was localized in livers from SD and PCK rats by picrosirius red staining. Images were taken at 100x magnification. A scale bar (100 μM) is included in each image. PV = portal vein, CV = central vein, and asterisks = cysts (some, but not all are indicated). (c) Inflammation in PCK rats. Monocyte chemoattractant protein-1 (MCP-1) transcripts in SD (blue circles) and PCK (red squares) rats from postnatal days (PND) 0 to 90 were measured in liver using real-time PCR as a surrogate marker for inflammation. The data are expressed as fold change over control (SD) at PND 0. All images are representative of = 2–4 rats at each time point and data are graphed as means plus standard error of the mean. between SD and PCK rats at the time points indicated.

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