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Activation of the unfolded protein response (UPR) is associated with cholangiocellular injury, fibrosis and carcinogenesis in an experimental model of fibropolycystic liver disease

Autores: Chen, C. B.; Wu, H. H.; Ye, H.; Tortajada, A.; Rodriguez-Perales, S.; Torres-Ruiz, R.; Vidal, A.; Peligros, M. I.; Reissing, J.; Bruns, T.; Mohamed, M. R.; Zheng, K.; Lujambio, A.; Iraburu Elizalde, María; Colyn, L.; Latasa Sada, María Ujué; Arechederra Calderon, Maria; García Fernández de Barrena, Maite; Berasain Lasarte, María del Carmen; Vaquero, J.; Banares, R.; Nelson, L. J.; Trautwein, C.; Davis, R. J.; Martínez-Naves, E.; Nevzorova, Y. A.; Villanueva, A.; Ávila Zaragoza, Matías Antonio (Autor de correspondencia); Cubero, F. J. (Autor de correspondencia)
Título de la revista: CANCERS
ISSN: 2072-6694
Volumen: 14
Número: 1
Páginas: 78
Fecha de publicación: 2022
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.