Of Cilia and Cysts: Modeling Pancreatic Polycystic Disease Klaus H. Kaestner  Gastroenterology  Volume 130, Issue 3, Pages 926-928 (March 2006) DOI: 10.1053/j.gastro.2006.01.056 Copyright © 2006 American Gastroenterological Association Terms and Conditions

Figure 1 Phenotype of the HNF-6 deficient pancreas. Schematic representation of acinar-ductal units normal (left) and HNF-6 null (right) pancreas. In wild-type pancreatic ducts, epithelial polarity is indicated by the presence of Mucin 1 (red) on the apical, and β-catenin (green) on the lateral surface of the cell. Each epithelial cell also contains a primary cilium. In the HNF-6 deficient pancreas, the intra-and interlobular ducts are abnormal, with the occurrence of multilayered epithelia with abnormal cellular polarity, the absence of primary cilia, and the formation of cysts. Illustration by Jerry Schoendorf, MAMS. Gastroenterology 2006 130, 926-928DOI: (10.1053/j.gastro.2006.01.056) Copyright © 2006 American Gastroenterological Association Terms and Conditions

Figure 2 Proposed transcription factor network controlling differentiation of intra- and interlobular ducts. HNF-6 is required for the activation of genes that establish normal epithelial polarity. In addition, HNF-6 also regulates components of the primary cilium like Cystin and Polyductin. While Cystin is likely controlled by HNF-6 directly, the activation of Polyductin involves a transcription factor cascade with HNF-1β. Illustration by Jerry Schoendorf, MAMS. Gastroenterology 2006 130, 926-928DOI: (10.1053/j.gastro.2006.01.056) Copyright © 2006 American Gastroenterological Association Terms and Conditions