Cholangiocyte autophagy contributes to hepatic cystogenesis in polycystic liver disease and represents a potential therapeutic target

• Published in: Hepatology (Baltimore, Md.) Vol. 67; no. 3; pp. 1088 - 1108
• Main Authors: Masyuk, Anatoliy I., Masyuk, Tatyana V., Lorenzo Pisarello, Maria J., Ding, Jingyi (Francess), Loarca, Lorena, Huang, Bing Q., LaRusso, Nicholas F.
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01.03.2018
• Subjects: Animals; Autophagy; Autophagy - drug effects; Autophagy - genetics; Autophagy - physiology; Bile Ducts - cytology; Bile Ducts - metabolism; Blotting, Western; Cell culture; Cell Proliferation - drug effects; Cells, Cultured; Cluster Analysis; Confocal microscopy; Cyclic AMP; Cysts; Cysts - drug therapy; Cysts - metabolism; Cysts - physiopathology; Disease Models, Animal; Enzyme Inhibitors - pharmacology; Epithelial Cells - metabolism; Female; Fluorescent Antibody Technique; Gene expression; Genetic disorders; Hepatology; High-Throughput Nucleotide Sequencing - methods; Humans; Hydroxychloroquine; Hydroxychloroquine - pharmacology; Immunofluorescence; Kidney diseases; Kidneys; Kinases; Liver - metabolism; Liver - pathology; Liver diseases; Liver Diseases - metabolism; Liver Diseases - physiopathology; Lysosomes; Male; Microscopy; Microscopy, Electron, Transmission; Morbidity; Phagocytosis; Phagosomes; Phenotypes; Polycystic kidney; Protein kinase A; Rats; Rats, Sprague-Dawley; Rodents; Signal Transduction; siRNA; Somatostatin - analogs & derivatives; Somatostatin - pharmacology; Transmission electron microscopy; Western blotting

Polycystic liver disease (PLD) is a group of genetic disorders with limited treatment options and significant morbidity. Hepatic cysts arise from cholangiocytes exhibiting a hyperproliferative phenotype. Considering that hyperproliferation of many cell types is associated with alterations in autopha...