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dc.creatorChen, C. (Chaobo)-
dc.creatorWu, H. (Hanghang)-
dc.creatorYe, H. (Hui)-
dc.creatorTortajada, A. (Agustín)-
dc.creatorRodriguez-Perales, S. (Sandra)-
dc.creatorTorres-Ruiz, R. (Raúl)-
dc.creatorVidal, A. (August)-
dc.creatorPeligros, M.I. (María Isabel)-
dc.creatorReissing, J. (Johanna)-
dc.creatorBruns, T. (Tony)-
dc.creatorMohamed, M.R. (Mohamed Ramadan)-
dc.creatorZheng, K. (Kang)-
dc.creatorLujambio, A. (Amaya)-
dc.creatorIraburu-Elizalde, M.J. (María José)-
dc.creatorColyn, L. (Leticia)-
dc.creatorLatasa, M.U. (María Ujué)-
dc.creatorArechederra, M. (María)-
dc.creatorGarcía-Fernández-de-Barrena, M. (Maite)-
dc.creatorBerasain, C. (Carmen)-
dc.creatorVaquero, J. (Javier)-
dc.creatorBañares, R. (Rafael)-
dc.creatorNelson, L.J. (Leonard J.)-
dc.creatorTrautwein, C. (Christian)-
dc.creatorDavis, R.J. (Roger J.)-
dc.creatorMartínez-Naves, E. (Eduardo)-
dc.creatorNevzorova, Y. (Yulia)-
dc.creatorVillanueva, A. (Alberto)-
dc.creatorAvila, M.A. (Matías Antonio)-
dc.creatorCubero, F.J. (Francisco Javier)-
dc.identifier.citationChen, 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, 78es
dc.description.abstractPolycystic 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.-
dc.description.sponsorshipThis research was funded by the MINECO Retos PID2020-117941RB-IOO, SAF2016-78711 and SAF2017-87919-R, MINECO Retos PID2019-104878RB-100AEI/10.13039/501100011033, EXOHEP-CM S2017/BMD-3727, NanoLiver-CM Y2018/NMT-4949, ERAB Ref. EA 18/14, AMMF 2018/117, UCM-25-2019, the German Research Foundation (SFB/TRR57/P04, SFB 1382-403224013/A02 and DFG NE 2128/2-1), and COST Action CA17112. T.B. was supported by the German Research Foundation (DFG) (SFB1382 Project ID 403114013/B07). C.C. is supported with a scientific research project of Wuxi Municipal Health Commission (M202160). L.J.N. is a recipient of a BBSRC (Grant BB/L023687/1) and a EPSRC (IAA Grant PIII013) grants. Y.A.N., M.G.F.B and F.J.C. are Ramón y Cajal Researcher RYC-2015-17438, RYC-2018-024475-1 and RYC-2014-15242, respectively. M.A. is a postdoctoral fellow of the Asociacion Española Contra el Cancer (AECC). L.C. is a predoctoral fellow of the Gobierno de Navarra. CT was supported by the German Research Foundation (DFG CRC1382 #403224013, TR285/10-2, GRK 2375). F.J.C. is a Gilead Liver Researcher 2018. The research group belongs to the validated Research Groups Ref. 970935 Liver Pathophysiology, 920631 Lymphocyte immunobiology, 920361 “Inmunogenética e inmunología de las mucosas” and IBL-6 (imas12-associated).-
dc.subjectc-Jun N-terminal kinases (JNK)-
dc.subjectFibropolycystic liver disease-
dc.subjectCholangiocarcinoma (CCA)-
dc.subjectEndoplasmic reticulum (ER)-
dc.subjectStressthioacetamide (TAA)-
dc.titleActivation of the unfolded protein response (UPR) is associated with cholangiocellular injury, fibrosis and carcinogenesis in an experimental model of fibropolycystic liver disease-
dc.description.noteThis 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/).-

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