Loss of CAPS2/Cadps2 leads to exocrine pancreatic cell injury and intracellular accumulation of secretory granules in mice by Yotaroh Sato, Miho Tsuyusaki, Hiromi Takahashi-Iwanaga, Rena Fujisawa, Atsushi Masamune, Shin Hamada, Ryotaro Matsumoto, Yu Tanaka, Yoichi Kakuta, Yumi Yamaguchi-Kabata, Tamio Furuse, Shigeharu Wakana, Takuya Shimura, Takuya Shimura, Rika Kobayashi, Yo Shinoda, Ryo Goitsuka, So Maezawa, Tetsushi Sadakata, Yoshitake Sano, Teiichi Furuichi

“…Caps2/Cadps2-knockout (KO) mice exhibited morphophysiological abnormalities in the exocrine pancreas, including excessive accumulation of secretory granules (zymogen granules) and their amylase content in the cytoplasm, deterioration of the fine intracellular membrane structures (disorganized rough endoplasmic reticulum, dilated Golgi cisternae, and the appearance of empty vesicles and autophagic-like vacuoles), as well as exocrine pancreatic cell injury, including acinar cell atrophy, increased fibrosis, and inflammatory cell infiltration. …”
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Clinical and genetic analysis of trichohepatoneurodevelopmental syndrome caused by a CCDC47 variant by Qi Yang, Xunzhao Zhou, Yeying Ling, Qiang Zhang, Shang Yi, Qiuli Chen, Shujie Zhang, Zailong Qin, Jingsi Luo

“…Trichohepatoneurodevelopmental syndrome is an extremely uncommon autosomal recessive disorder resulting from variants in the CCDC47 gene, which encodes a Ca2+-binding endoplasmic reticulum (ER) transmembrane protein. To date, only four patients with CCDC47 deficiency have been reported, all of them with homozygous truncating CCDC47 variants. …”
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α-Linolenic Acid-Enriched Cold-Pressed Perilla Oil Suppress High-Fat Diet-Induced Hepatic Steatosis through Amelioration of the ER Stress-Mediated Autophagy by Su Ji Bae, Ji Eun Kim, Hyeon Jun Choi, Yun Ju Choi, Su Jin Lee, Jeong Eun Gong, Sungbaek Seo, Seung Yun Yang, Beum-Soo An, Hee Seob Lee, Dong Seob Kim, Chung Yeoul Lee, Dae Youn Hwang

“…The therapeutic effects and molecular mechanism of an α-linolenic acid-enriched cold-pressed perilla oil (LEP) on hepatic steatosis of an obesity model were investigated by analyzing alterations in fat accumulation and endoplasmic reticulum (ER) stress-mediated autophagy, in high-fat diet (HFD)-induced obesity C57BL/6N mice treated with LEP for 16 weeks. …”
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Independent activation of CREB3L2 by glucose fills a regulatory gap in mouse β-cells by co-ordinating insulin biosynthesis with secretory granule formation by Nancy Sue, Le May Thai, Atsushi Saito, Cierra K. Boyer, Ashleigh M. Fordham, Chenxu Yan, Aimee Davenport, Jiang Tao, Mohammed Bensellam, James Cantley, Yan-Chuan Shi, Samuel B. Stephens, Kazunori Imaizumi, Trevor J. Biden

“…Objective: Although individual steps have been characterized, there is little understanding of the overall process whereby glucose co-ordinates the biosynthesis of insulin with its export out of the endoplasmic reticulum (ER) and incorporation into insulin secretory granules (ISGs). …”
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C9orf72 gene networks in the human brain correlate with cortical thickness in C9-FTD and implicate vulnerable cell types by Iris J. Broce, Iris J. Broce, Daniel W. Sirkis, Ryan M. Nillo, Luke W. Bonham, Luke W. Bonham, Suzee E. Lee, Bruce L. Miller, Patricia A. Castruita, Virginia E. Sturm, Virginia E. Sturm, Leo S. Sugrue, Rahul S. Desikan, Jennifer S. Yokoyama, Jennifer S. Yokoyama, Jennifer S. Yokoyama

“…This C9orf72 imaging transcriptomic network was differentially expressed in cell populations previously implicated in ALS and FTD, including layer 5b cells, cholinergic neurons in the spinal cord and brainstem and medium spiny neurons of the striatum, and was enriched for biological and molecular pathways associated with protein ubiquitination, autophagy, cellular response to DNA damage, endoplasmic reticulum to Golgi vesicle-mediated transport, among others.ConclusionConsidered together, we identified a network of C9orf72 associated genes that may influence selective regional and cell-type-specific vulnerabilities in ALS/FTD.…”
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miR-18a-5p Is Involved in the Developmental Origin of Prostate Cancer in Maternally Malnourished Offspring Rats: A DOHaD Approach by Sergio Alexandre Alcantara Santos, Luiz Marcos Frediani Portela, Ana Carolina Lima Camargo, Flavia Bessi Constantino, Ketlin Thassiani Colombelli, Matheus Naia Fioretto, Renato Mattos, Bruno Evaristo de Almeida Fantinatti, Michela Alessandra Denti, Silvano Piazza, Sérgio Luis Felisbino, Elena Zambrano, Luis Antonio Justulin

“…Integrative analysis of the (VP) transcriptomic and proteomic data revealed changes in the expression profile of 14 identified predicted targets of these two DE miRNAs, which enriched terms related to post-translational protein modification, metabolism of proteins, protein processing in endoplasmic reticulum, phosphonate and phosphinate metabolism, the calnexin/calreticulin cycle, metabolic pathways, N-glycan trimming in the ER and the calnexin/calreticulin cycle, hedgehog ligand biogenesis, the ER-phagosome pathway, detoxification of reactive oxygen species, antigenprocessing-cross presentation, RAB geranylgeranylation, collagen formation, glutathione metabolism, the metabolism of xenobiotics by cytochrome P450, and platinum drug resistance. …”
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Hepatocyte-specific mitogen-activated protein kinase phosphatase 1 in sexual dimorphism and susceptibility to alcohol induced liver injury by Mary Nancy Walter, Diego Montoya-Durango, Walter Rodriguez, Yali Wang, JingWen Zhang, Julia H. Chariker, Julia H. Chariker, Eric C. Rouchka, Eric C. Rouchka, Claudio Maldonado, Anton Bennett, Craig James McClain, Craig James McClain, Craig James McClain, Craig James McClain, Craig James McClain, Shirish Barve, Shirish Barve, Shirish Barve, Leila Gobejishvili, Leila Gobejishvili, Leila Gobejishvili, Leila Gobejishvili

“…Statistical analysis was carried out using ANOVA and the unpaired Student’s t-test.ResultsASH was associated with the severe injury accompanied by increased endoplasmic reticulum (ER) stress and significant downregulation of Dusp1 mRNA expression. …”
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Combination Therapy with STAT3 Inhibitor Enhances SERCA2a-Induced BMPR2 Expression and Inhibits Pulmonary Arterial Hypertension by Malik Bisserier, Michael G. Katz, Carlos Bueno-Beti, Agnieszka Brojakowska, Shihong Zhang, Sarah Gubara, Erik Kohlbrenner, Shahood Fazal, Anthony Fargnoli, Peter Dorfmuller, Marc Humbert, Akiko Hata, David A. Goukassian, Yassine Sassi, Lahouaria Hadri

“…Here, we found that decreased expression of sarcoplasmic/endoplasmic reticulum Ca²⁺ ATPase 2a (SERCA2a) in the lung samples of PAH patients was associated with the down-regulation of bone morphogenetic protein receptor type 2 (BMPR2) and the activation of signal transducer and activator of transcription 3 (STAT3). …”
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Mitochondria-associated membrane protein PACS2 maintains right cardiac function in hypobaric hypoxia by Jie Yang, Mengjia Sun, Renzheng Chen, Xiaowei Ye, Boji Wu, Zhen Liu, Jihang Zhang, Xubin Gao, Ran Cheng, Chunyan He, Jingyu He, Xuhong Wang, Lan Huang

“…Cardiomyocyte Pacs2 deficiency disrupted MAM formation and endoplasmic reticulum (ER)-mitochondria calcium flux, further inhibiting mitophagy and energy metabolism in HH. …”
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ER stress mediates Angiotensin II-augmented innate immunity memory and facilitates distinct susceptibilities of thoracic from abdominal aorta to aneurysm development by Yifan Lu, Yu Sun, Fatma Saaoud, Ying Shao, Keman Xu, Xiaohua Jiang, Xiaohua Jiang, Sheng Wu, Jun Yu, Nathaniel W. Snyder, Ling Yang, Xinghua Mindy Shi, Huaqing Zhao, Hong Wang, Xiaofeng Yang, Xiaofeng Yang

“…To determine the roles of endoplasmic reticulum (ER) stress and trained immunity, we performed transcriptome analyses on the thoracic aorta (TA) and abdominal aorta (AA) from the angiotensin II (Ang II)-HFD-ApoE-KO aneurysm model and made significant findings: 1) Ang II bypassed HFD-induced metabolic reprogramming and induced stronger inflammation in AA than in TA; 2) Ang II and HFD upregulated 890 genes in AA versus TA and induced cytokine signaling; 3) Ang II AA and TA upregulated 73 and 68 cytokines, scRNA-Seq identified markers of macrophages and immune cells, cell death regulators, respectively; transdifferentiation markers of neuron, glial, and squamous epithelial cells were upregulated by Ang II-AA and TA; and pyroptosis signaling with IL-1β and caspase-4 were more upregulated in Ang II-AA than in TA; 4) Six upregulated transcriptomes in patients with AAA, Ang II AA, Ang II TA, additional aneurysm models, PPE-AAA and BAPN-Ang II-AAA, were partially overlapped with 10 lists of new ER stress gene sets including 3 interaction protein lists of ER stress regulators ATF6, PERK, and IRE1, HPA ER localization genes, KEGG signal genes, XBP1 transcription targets, ATF4 (PERK) targets, ATF6 targets, thapsigargin ER stress genes, tunicamycin-ER stress genes, respectively; 5) Ang II-AA and TA upregulated ROS regulators, MitoCarta genes, trained immunity genes, and glycolysis genes; and 6) Gene KO transcriptomes indicated that ATF6 and PERK played more significant roles than IRE1 in promoting AAA and trained immunity whereas antioxidant NRF2 inhibited them. …”
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DDRGK1-mediated ER-phagy attenuates acute kidney injury through ER-stress and apoptosis by Haijiao Jin, Yuanting Yang, Xuying Zhu, Yin Zhou, Yao Xu, Jialin Li, Chaojun Qi, Xinghua Shao, Jingkui Wu, Shan Wu, Hong Cai, Leyi Gu, Shan Mou, Zhaohui Ni, Shu Li, Qisheng Lin

“…This study investigates the interplay between endoplasmic reticulum (ER) stress, unfolded protein response (UPR), and ER-associated degradation (ER-phagy) in the pathogenesis of AKI. …”
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PLAAT1 inhibits type I interferon response via degradation of IRF3 and IRF7 in Zebrafish by Xin Zhao, Xin Zhao, Xin Zhao, Wenji Huang, Wenji Huang, Wenji Huang, Yanjie Shi, Yanjie Shi, Yanjie Shi, Jiahong Guo, Jiahong Guo, Jiahong Guo, Hehe Xiao, Hehe Xiao, Hehe Xiao, Ning Ji, Ning Ji, Ning Ji, Jianhua Feng, Jianhua Feng, Jianhua Feng, Huifeng Dang, Huifeng Dang, Huifeng Dang, Jun Zou, Jun Zou, Jun Zou, Jun Zou

“…Recently, PLAAT1 has been shown to promote degradation of p53 protein and cellular organelles such as mitochondria, endoplasmic reticulum and lysosome. In this study, we show that PLAAT1 inhibits the production of type I interferon and promotes virus replication in zebrafish. …”
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Alzheimer and Parkinson diseases, frontotemporal lobar degeneration and amyotrophic lateral sclerosis overlapping neuropathology start in the first two decades of life in pollution... by Lilian Calderón-Garcidueñas, Elijah W. Stommel, Ricardo Torres-Jardón, Jacqueline Hernández-Luna, Mario Aiello-Mora, Angélica González-Maciel, Rafael Reynoso-Robles, Beatriz Pérez-Guillé, Héctor G. Silva-Pereyra, Samuel Tehuacanero-Cuapa, Arturo Rodríguez-Gómez, Ingolf Lachmann, Carolina Galaz-Montoya, Richard L. Doty, Anik Roy, Partha S. Mukherjee

“…Endothelial, neuronal, and glial damages are extensive, with NPs in mitochondria, rough endoplasmic reticulum, the Golgi apparatus, and lysosomes. …”
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Stromal Interaction Molecule 1 Promotes the Replication of vvIBDV by Mobilizing Ca²⁺ in the ER by Nana Yan, Yongqiang Wang, Zehua Chen, Aijing Liu, Yue Li, Bo Yang, Kai Li, Xiaole Qi, Yulong Gao, Li Gao, Changjun Liu, Yanping Zhang, Hongyu Cui, Qing Pan, Xiaomei Wang

“…In our study, we found Ca²⁺ levels in the endoplasmic reticulum (ER) of vvIBDV-infected B cells were higher than that of mock-infected cells, and the expression level of stromal interaction molecule 1 (STIM1), an ER Ca²⁺ sensor, was significantly upregulated due to vvIBDV infection. …”
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Activating mutations in JAK2 and CALR differentially affect intracellular calcium flux in store operated calcium entry by Vikas Bhuria, Tobias Franz, Conny Baldauf, Martin Böttcher, Nicolas Chatain, Steffen Koschmieder, Tim H. Brümmendorf, Dimitrios Mougiakakos, Burkhart Schraven, Sascha Kahlfuß, Thomas Fischer

“…The impact of JAK2-V617F and CALR mutations which are disease initiating in myeloproliferative neoplasms (MPN) on SOCE, calcium flux from the endoplasmic reticulum (ER) to the cytosol, and related key signaling pathways in the presence or absence of erythropoietin (EPO) or thrombopoietin (TPO) is poorly understood. …”
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The Prognostic Significance of <i>FKBP1A</i> and Its Related Immune Infiltration in Liver Hepatocellular Carcinoma by Zhongguang Li, Ying Cui, Qinchun Duan, Jianfei Zhang, Danyang Shao, Xixi Cao, Yuru Gao, Shulin Wang, Jiali Li, Odell D. Jones, Xinjuan Lei, Liyang Wang, Xin Zhou, Mengmeng Xu, Jianjie Ma, Yingli Liu, Xuehong Xu

“…The expression level of the FKBP1A protein was also increased in LIHC patients along with its accumulation in endoplasmic reticulum (ER). High <i>FKBP1A</i> expression was correlated with a poor survival rate in LIHC patients. …”
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Predictive and Prognostic Brain Metastases Assessment in Luminal Breast Cancer Patients: FN14 and GRP94 from Diagnosis to Prophylaxis by Antonio Martínez-Aranda, Antonio Martínez-Aranda, Vanessa Hernández, Ferran Moreno, Núria Baixeras, Daniel Cuadras, Ander Urruticoechea, Miguel Gil-Gil, Noemí Vidal, Xavier Andreu, Miquel A. Seguí, Rosa Ballester, Eva Castella, Angels Sierra, Angels Sierra

“…FN14 has been implicated in many intracellular signaling pathways, and GRP94 is a well-known endoplasmic reticulum protein regulated by glucose. Recently, both have been associated with metastasis progression in breast cancer patients. …”
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A Multimodal Omics Exploration of the Motor and Non-Motor Symptoms of Parkinson’s Disease by François-Xavier Lejeune, Farid Ichou, Etienne Camenen, Benoit Colsch, Florence Mauger, Caroline Peltier, Ivan Moszer, Emmanuel Gilson, Morgane Pierre-Jean, Edith Le Floch, Victor Sabarly, Arthur Tenenhaus, Jean-François Deleuze, Claire Ewenczyk, Marie Vidailhet, Fanny Mochel

“…Furthermore, emotion variables were associated with phosphatidylcholines, cholesteryl esters and genes related to endoplasmic reticulum stress and cell regulation.…”
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A stress sensor, IRE1α, is required for bacterial-exotoxin-induced interleukin-1β production in tissue-resident macrophages by Izumi Sasaki, Yuri Fukuda-Ohta, Chihiro Nakai, Naoko Wakaki-Nishiyama, Chizuyo Okamoto, Daisuke Okuzaki, Shuhei Morita, Shiori Kaji, Yuki Furuta, Hiroaki Hemmi, Takashi Kato, Asumi Yamamoto, Emi Tosuji, Shin-Ichiroh Saitoh, Takashi Tanaka, Katsuaki Hoshino, Shinji Fukuda, Kensuke Miyake, Etsushi Kuroda, Ken J. Ishii, Takao Iwawaki, Koichi Furukawa, Tsuneyasu Kaisho

“…Here, we clarify the roles of inositol-requiring enzyme 1 alpha (IRE1α), an endoplasmic reticulum (ER) stress sensor, in CT-induced IL-1β production in RPMs. …”
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Artichoke (Cynara scolymus L.) water extract alleviates palmitate-induced insulin resistance in HepG2 hepatocytes via the activation of IRS1/PI3K/AKT/FoxO1 and GSK-3β signaling pat... by Aihua Deng, Yun Wang, Kerui Huang, Peng Xie, Ping Mo, Fengying Liu, Jun Chen, Kaiyi Chen, Yun Wang, Bing Xiao

“…Furthermore, the protective effect of AWE on IR HepG2 cells might be ascribed to the inhibition of the endoplasmic reticulum (ER) stress. Conclusion We conclude that AWE may improve glucose metabolism by regulating IRS1/PI3K/AKT/FoxO1 and GSK-3β signaling associated with the inhibition of ER stress in IR HepG2 cells induced by PA.…”
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