Activation of the unfolded protein response (UPR) is associated with cholangiocellular injury, fibrosis and carcinogenesis in an experimental model of fibropolycystic liver disease
Keywords: 
c-Jun N-terminal kinases (JNK)
Fibropolycystic liver disease
Cholangiocarcinoma (CCA)
Endoplasmic reticulum (ER)
Stressthioacetamide (TAA)
CM272
Issue Date: 
2022
ISSN: 
2072-6694
Note: 
This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
Citation: 
Chen, C. B.; Wu, H. H.; Ye, H.; et al. "Activation of the unfolded protein response (UPR) is associated with cholangiocellular injury, fibrosis and carcinogenesis in an experimental model of fibropolycystic liver disease". Cancers. 14 (1), 2022, 78
Abstract
Polycystic liver disease (PLD) is a group of rare disorders that result from structural changes in the biliary tree development in the liver. In the present work, we studied alterations in molecular mechanisms and signaling pathways that might be responsible for these pathologies. We found that activation of the unfolded protein response, a process that occurs in response to an accumulation of unfolded or misfolded proteins in the lumen of the endoplasmic reticulum, as well as the scarring of the liver tissue, contribute to the pathogenesis of PLD and the development of cancer. As a preclinical animal model we have used mutant mice of a specific signaling pathway, the c-Jun N-terminal kinase 1/2 (Jnk1/2). These mice resemble a perfect model for the study of PLD and early cancer development.

Files in This Item:
Thumbnail
File
pdf.pdf
Description
Size
20.41 MB
Format
Adobe PDF


Items in Dadun are protected by copyright, with all rights reserved, unless otherwise indicated.