Pathways dysregulated in PLD. Transcriptome profiling of NHCs and PLDCs revealed that out of 11,897 transcripts present in both cell lines, 6,500 transcripts were differentially expressed in PLDCs (i.e., up- or downregulated). Functional annotation clustering of these transcripts by the Kyoto Encyclopedia of Genes and Genomes pathways indicates 30 altered pathways in PLDCs. Abbreviations: cAMP, cyclic adenosine monophosphate; CDC25, cell division cycle 25; CREB, cAMP-response element-binding protein; ECM, extracellular matrix; EGF, endothelial growth factor; ERK, extracellular signal-regulated kinase; MAP, mitogen-activated protein; mTOR mammalian target of rapamycin; NHC, normal human cholangiocyte; PI3, phosphoinositide 3; PLD, polycystic liver disease; PLDC, polycystic liver disease cholangiocyte; TGF, transforming growth factor; VEGF, vascular endothelial growth factor. Scanning electron microscope image adapted with permission from Reference 22.