Alliance Supported Research Update

With the support of the ARPKD/CHF Alliance, work in my lab on the mechanism of CHF has made considerable progress over the last six months. We are collaborating with groups in Birmingham, AL, Cleveland, OH, and Irvine, CA.  We have identified the fibroblast cell population responsible for fibrosis in CHF and have strong evidence that these cells become fibrogenic only in response to abnormal signaling from biliary epithelial cells, the cells in the liver expressing fibrocystin (the protein that is abnormal in ARPKD/CHF).

Jessica Wen, MD, a fellow in Gastroenterology, Hepatology, and Nutrition from the Children’s Hospital of Philadelphia, is studying the signals traveling between biliary epithelial cells and fibroblasts.  She has identified several candidate proteins that may be secreted abnormally from biliary epithelial cells in ARPKD/CHF and may cause abnormal activation of fibroblasts with resulting fibrosis.  Work in the lab in the next six months will focus on characterizing these proteins in various cell culture systems. We hope our findings will ultimately point towards new treatments for liver fibrosis in ARPKD/CHF.