α-Gal A missense variants associated with Fabry disease can lead to ER stress and induction of the unfolded protein response by Francesco Consolato, Maurizio De Fusco, Céline Schaeffer, Federico Pieruzzi, Francesco Scolari, Maurizio Gallieni, Chiara Lanzani, Sandro Feriozzi, Luca Rampoldi

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The Peptide–Drug Conjugate Melflufen Modulates the Unfolded Protein Response of Multiple Myeloma and Amyloidogenic Plasma Cells and Induces Cell Death by Ken Flanagan, Romika Kumari, Juho J. Miettinen, Staci L. Haney, Michelle L. Varney, Jacob T. Williams, Muntasir M. Majumder, Minna Suvela, Ana Slipicevic, Fredrik Lehmann, Nina N. Nupponen, Sarah A. Holstein, Caroline A. Heckman

“…We describe the effects of melflufen on amyloidogenic plasma cells in vitro and ex vivo, demonstrating enhanced cytotoxic effects in comparison to melphalan, as well as novel mechanisms of action through the unfolded protein response (UPR) pathway. These findings provide evidence that melflufen-mediated cytotoxicity extends to amyloidogenic plasma cells, and support the rationale for the evaluation of melflufen in patients with AL amyloidosis.…”
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The Unfolded Protein Response Sensor IRE1 Regulates Activation of In Vitro Differentiated Type 1 Conventional DCs with Viral Stimuli by Bernardita Medel, José Ignacio Bernales, Alonso Lira, Dominique Fernández, Takao Iwawaki, Pablo Vargas, Fabiola Osorio

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The Effect of Resistance Training and Berberine Chloride on the Apoptosis-related Unfolded Protein Response Signaling Pathway in the Hippocampus of Diazinon-poisoned Rats by Ali Esfandiarifar, Mohammad Ali Azarbayjani, Maghsood Peeri, Seyed Behnamedin Jameie

“…It has been proved that activation of Unfolded Protein Response (UPR) under oxidative stress is a steady mechanism for maintaining cell function and survival. …”
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<i>Arabidopsis</i> Defense against the Pathogenic Fungus <i>Drechslera gigantea</i> Is Dependent on the Integrity of the Unfolded Protein Response by Simone Samperna, Angela Boari, Maurizio Vurro, Anna Maria Salzano, Pierluigi Reveglia, Antonio Evidente, Angelo Gismondi, Antonella Canini, Andrea Scaloni, Mauro Marra

“…<i>Drechslera gigantea</i> Heald & Wolf is a worldwide-spread necrotrophic fungus closely related to the <i>Bipolaris</i> genus, well-known because many member species provoke severe diseases in cereal crops and studied because they produce sesterpenoid phytoxins named ophiobolins which possess interesting biological properties. The unfolded protein response (UPR) is a conserved mechanism protecting eukaryotic cells from the accumulation of unfolded/misfolded proteins in the endoplasmic reticulum (ER). …”
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Unfolded protein response pathways in stroke patients: a comprehensive landscape assessed through machine learning algorithms and experimental verification by Haiyang Yu, Xiaoyu Ji, Yang Ouyang

“…Abstract Background The unfolding protein response is a critical biological process implicated in a variety of physiological functions and disease states across eukaryotes. …”
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AMPK Mediates Early Activation of the Unfolded Protein Response through a Positive Feedback Loop in Palmitate-Treated Muscle Cells by Jing Gong, Lu Wang, Wuchen Tao, Xiangsheng Pang, Zonghan Liu, Shiming Li, Wenjiong Li, Xiaoping Chen, Peng Zhang

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The Inflammatory Bowel Disease Drug Azathioprine Induces Autophagy via mTORC1 and the Unfolded Protein Response Sensor PERK by Hooper, K, Casanova, V, Kemp, S, Staines, K, Satsangi, J, Barlow, P, Henderson, P, Stevens, C

“…RESULTS:Azathioprine induces autophagy via mechanisms involving modulation of mechanistic target of rapamycin (mTORC1) signaling and stimulation of the unfolded protein response (UPR) sensor PERK. Induction of autophagy with azathioprine correlated with the enhanced clearance of AIEC and dampened AIEC-induced increases in TNFα. …”

Synergistic lethality in chronic myeloid leukemia – targeting oxidative phosphorylation and unfolded protein response effectively complements tyrosine kinase inhibitor treatment by Lukas Häselbarth, Sara Gamali, Domenica Saul, Manuela Krumbholz, Romy Böttcher-Loschinski, Martin Böttcher, Deyu Zou, Markus Metzler, Axel Karow, Dimitrios Mougiakakos

“…Preliminary studies have shown that targeting oxidative phosphorylation (oxPhos) and the unfolded protein response (UPR) are promising therapeutic approaches to complement CML treatment. …”
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Congenital Tufting Enteropathy-Associated Mutant of Epithelial Cell Adhesion Molecule Activates the Unfolded Protein Response in a Murine Model of the Disease by Barun Das, Kevin Okamoto, John Rabalais, Ronald R. Marchelletta, Kim E. Barrett, Soumita Das, Maho Niwa, Mamata Sivagnanam

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LINP1 represses unfolded protein response by directly inhibiting eIF2α phosphorylation to promote cutaneous squamous cell carcinoma by Xiaoting Liang, Jieyu Liu, Xingyuan Liu, Yi Jin, Minna Xu, Zhenyu Han, Ke Wang, Chunting Zhang, Fei Zou, Liang Zhou

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IRE1-Mediated Unfolded Protein Response Promotes the Replication of Tick-Borne Flaviviruses in a Virus and Cell-Type Dependent Manner by Veronika J. M. Breitkopf, Gerhard Dobler, Peter Claus, Hassan Y. Naim, Imke Steffen

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DECIPHERING THE NOXIOUS INTERPLAY BETWEEN THE UNFOLDED PROTEIN RESPONSE AND ANTIOXIDANT RESPONSES UNDER ABERRANT METABOLIC STIMULI ASSOCIATED WITH DOWN SYNDROME NEUROPATHOLOGY by Fabio Di Domenico, Chiara Lanzillotta, Francesca Prestia, Antonella Tramutola, Marzia Perluigi, Eugenio Barone

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Kisspeptin-10 Improves Testicular Redox Status but Does Not Alter the Unfolded Protein Response (UPR) That Is Downregulated by Hypothyroidism in a Rat Model by Luciano Cardoso Santos, Jeane Martinha dos Anjos Cordeiro, Maria Clara da Silva Galrão Cunha, Bianca Reis Santos, Luciana Santos de Oliveira, Adriana Lopes da Silva, Erikles Macêdo Barbosa, Raquel Vieira Niella, Gustavo José Cota de Freitas, Daniel de Assis Santos, Rogéria Serakides, Natália de Melo Ocarino, Stephanie Carvalho Borges, Mário Sérgio Lima de Lavor, Juneo Freitas Silva

“…In this study, we evaluate the effects of kisspeptin-10 (Kp10) on the testicular redox, as well as mediators of the unfolded protein response (UPR) in adult rats with hypothyroidism. …”
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Effects of Maternal Grape Juice Intake on Unfolded Protein Response in the Mammary Glands of Offspring of High Fat Diet Fed Rat Dams by Caroline Dani, Luciana Kneib Gonçalves, Isabel Teixeira Proença, Fabia de Oliveira Andrade, Leena Hilakivi-Clarke

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Pan-Sigma Receptor Modulator RC-106 Induces Terminal Unfolded Protein Response In <i>In Vitro</i> Pancreatic Cancer Model by Michela Cortesi, Alice Zamagni, Sara Pignatta, Michele Zanoni, Chiara Arienti, Daniela Rossi, Simona Collina, Anna Tesei

“…Their main localization suggests they play a potential role in ER stress and in the triggering of the unfolded protein response (UPR). Here, we investigated the mechanisms of action of <b>RC-106</b>, a novel pan-SR modulator, to characterize therapeutically exploitable role of SRs in tumors. …”
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CRISPR screen identifies the role of RBBP8 in mediating unfolded protein response induced liver damage through regulating protein synthesis by Heting Wang, Xuya Pan, Xiaoxin Xiang, Yang Zhang, Jianning Chen, Shiyi Wen, Jin Wang, Rong Gao, Jifeng Yang, Yaping Zhi, Siying Wen, Yubao Zheng, Ting Li, Heying Ai, Xuemin He, Yan Lu, Yanhua Zhu, Chunliang Li, Yanming Chen, Guojun Shi

“…Abstract Unfolded protein response (UPR) maintains the endoplasmic reticulum (ER) homeostasis, survival, and physiological function of mammalian cells. …”
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Arsenic Activates the ER Stress-Associated Unfolded Protein Response via the Activating Transcription Factor 6 in Human Bronchial Epithelial Cells by Priya Wadgaonkar, Zhuoyue Bi, Junmei Wan, Yao Fu, Qian Zhang, Bandar Almutairy, Wenxuan Zhang, Yiran Qiu, Chitra Thakur, Maik Hüttemann, Fei Chen

“…In the present report, we investigate the short-term effect of As³⁺ on the endoplasmic reticulum (ER) stress response—the “unfolded protein response (UPR)” and metabolism in human bronchial epithelial cell line BEAS-2B cells. …”
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Macrophage Reprogramming via the Modulation of Unfolded Protein Response with siRNA-Loaded Magnetic Nanoparticles in a TAM-like Experimental Model by Annarita D’Urso, Francesca Oltolina, Chiara Borsotti, Maria Prat, Donato Colangelo, Antonia Follenzi

“…TAMs display an irregular unfolded protein response (UPR) in their endoplasmic reticulum (ER) to endure environmental stress and ensure anti-cancer immunity. …”
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Targeting Glioblastoma Stem Cells with 2-Deoxy-D-Glucose (2-DG) Potentiates Radiation-Induced Unfolded Protein Response (UPR) by Sumedh S. Shah, Gregor A. Rodriguez, Alexis Musick, Winston M. Walters, Nicolas de Cordoba, Eric Barbarite, Megan M. Marlow, Brian Marples, Jeffrey S. Prince, Ricardo J. Komotar, Steven Vanni, Regina M. Graham

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