Mannose Phosphate Isomerase and Mannose Regulate Hepatic Stellate Cell Activation and Fibrosis in Zebrafish and Humans

• Published in: Hepatology (Baltimore, Md.) Vol. 70; no. 6; pp. 2107 - 2122
• Main Authors: DeRossi, Charles, Bambino, Kathryn, Morrison, Joshua, Sakarin, Isabel, Villacorta‐Martin, Carlos, Zhang, Changwen, Ellis, Jillian L., Fiel, M. Isabel, Ybanez, Maria, Lee, Youngmin A., Huang, Kuan‐lin, Yin, Chunyue, Sakaguchi, Takuya F., Friedman, Scott L., Villanueva, Augusto, Chu, Jaime
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01.12.2019
• Subjects: Animals; Cell activation; Cell culture; Cells, Cultured; Danio rerio; Ethanol; Fatty liver; Fibrosis; Gene expression; Glycosylation; Hepatic Stellate Cells - physiology; Hepatitis B; Hepatology; Humans; Liver Cirrhosis - etiology; Liver diseases; Male; Mannose; Mannose - pharmacology; Mannose-6-Phosphate Isomerase - physiology; Metabolism; Mpi gene; Platelet-Derived Growth Factor - physiology; Signal Transduction - physiology; Supplements; Zebrafish

The growing burden of liver fibrosis and lack of effective antifibrotic therapies highlight the need for identification of pathways and complementary model systems of hepatic fibrosis. A rare, monogenic disorder in which children with mutations in mannose phosphate isomerase (MPI) develop liver fibr...