Results 71 to 80 of about 1,902,157 (395)

Elexacaftor/Tezacaftor/Ivacaftor Efficacy in a Cohort of Italian Patients with CFTR Rare Mutations

International Journal of Translational Medicine
Background: Cystic Fibrosis is an inherited disorder caused by mutations in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) gene, encoding a chloride and bicarbonate channel widely expressed in epithelia.
Francesca Lucca, Sonia Volpi, Mirco Ros, Benedetta Fabrizzi, Ilaria Meneghelli, Marica Bordicchia, Francesca Buniotto, Alessia Lancini, Cecilia Brignole, Francesca Pauro, Valentino Bezzerri, Marco Cipolli   +11 more
doaj   +1 more source

A small molecule that induces translational readthrough of CFTR nonsense mutations by eRF1 depletion

Nature Communications, 2021
Premature termination codons can cause early translation termination and lead to disease. Here the authors perform a screen to identify compounds with readthrough activity and show that these reduce eRF1 levels to suppress premature termination ...
Jyoti Sharma, Ming Du, Eric Wong, Venkateshwar Mutyam, Yao Li, Jianguo Chen, Jamie Wangen, Kari Thrasher, Lianwu Fu, Ning Peng, Liping Tang, Kaimao Liu, Bini Mathew, Robert J. Bostwick, Corinne E. Augelli-Szafran, Hermann Bihler, Feng Liang, Jerome Mahiou, Josef Saltz, Andras Rab, Jeong Hong, Eric J. Sorscher, Eric M. Mendenhall, Candice J. Coppola, Kim M. Keeling, Rachel Green, Martin Mense, Mark J. Suto, Steven M. Rowe, David M. Bedwell   +29 more
doaj   +1 more source

Dammarenediol II enhances etoposide‐induced apoptosis by targeting O‐GlcNAc transferase and Akt/GSK3β/mTOR signaling in liver cancer

Molecular Oncology, EarlyView.
Etoposide induces DNA damage, activating p53‐dependent apoptosis via caspase‐3/7, which cleaves PARP1. Dammarenediol II enhances this apoptotic pathway by suppressing O‐GlcNAc transferase activity, further decreasing O‐GlcNAcylation. The reduction in O‐GlcNAc levels boosts p53‐driven apoptosis and influences the Akt/GSK3β/mTOR signaling pathway ...
Jaehoon Lee, Byung‐Cheol Han, Gi‐Bang Koo, Jihye Park, Mijin Kwon, Young Bin Park, Jae‐Mun Choi, Seung‐Ho Lee, Sangho Roh   +8 more
wiley   +1 more source

Sequential Wnt Agonist then Antagonist Treatment Accelerates Tissue Repair and Minimizes Fibrosis [PDF]

, 2019
Tissue fibrosis compromises organ function and occurs as a potential long-term outcome in response to acute tissue injuries. Currently, lack of mechanistic understanding prevents effective prevention and treatment of the progression from acute injury to ...
Fu, Haiyan, Huang, Sui, Liu, Youhua, Tian, Xiao-Jun, Wang, Xiaojie, Xing, Jianhua, Zhang, Rong, Zhou, Dong   +7 more
core   +2 more sources

Characterisation of feline renal cortical fibroblast cultures and their transcriptional response to transforming growth factor beta 1 [PDF]

, 2018
Chronic kidney disease (CKD) is common in geriatric cats, and the most prevalent pathology is chronic tubulointerstitial inflammation and fibrosis. The cell type predominantly responsible for the production of extra-cellular matrix in renal fibrosis is ...
A Clayton, A Desmouliere, A Holmes, A Yabuki, AA Eddy, AA Eddy, AC Sanchez-Lara, BL Aken, C Grupp, C. P. D. Wheeler-Jones, CH Heldin, CL Marino, E Honda, E Van Obberghen-Schilling, F Strutz, F Strutz, F Syeda, GR Grotendorst, H. M. Syme, HP Rodemann, J Ivaska, J Lu, J. Elliott, J. S. Lawson, JL Mumaw, JS Lawson, K Sawashima, KG McCullagh, KJ Kelynack, LC Penning, M Prunotto, M Zeisberg, MA Venkatachalam, MJ Ryan, MK Phanish, ML Sequeira Lopez, P Boor, P Sysa, PC Bartlett, Q Chai, RA Ignotz, S Chakrabarti, S Meran, S Moll, S Neelisetty, SC Chen, SL Lin, SP DiBartola, SW Smith, T Akamatsu, TA Wynn, V Dugina, VS Lebleu, W Bloom, WA Border, X Pan, XM Meng, Y Hosokawa, Y Liu   +58 more
core   +2 more sources

Association Between Fibrosis Stage and Outcomes of Patients with Non-Alcoholic Fatty Liver Disease: a Systematic Review and Meta-Analysis.

Gastroenterology, 2020
BACKGROUND & AIMS Biopsy-confirmed liver fibrosis is a prognostic factor for patients with non-alcoholic fatty liver disease (NAFLD). We performed a systematic review to quantify the prognostic value of fibrosis stage in patients with NAFLD and the ...
R. S. Taylor, R. J. Taylor, Sue Bayliss, H. Hagström, Patrik Nasr, J. Schattenberg, M. Ishigami, H. Toyoda, V. Wai‐Sun Wong, N. Peleg, A. Shlomai, G. Sebastiani, Yuya Seko, N. Bhala, Z. Younossi, Q. Anstee, S. McPherson, P. Newsome   +17 more
semanticscholar   +1 more source

COMP–PMEPA1 axis promotes epithelial‐to‐mesenchymal transition in breast cancer cells

Molecular Oncology, EarlyView.
This study reveals that cartilage oligomeric matrix protein (COMP) promotes epithelial‐to‐mesenchymal transition (EMT) in breast cancer. We identify PMEPA1 (protein TMEPAI) as a novel COMP‐binding partner that mediates EMT via binding to the TSP domains of COMP, establishing the COMP–PMEPA1 axis as a key EMT driver in breast cancer.
Konstantinos S. Papadakos, Gilar Gorji‐bahri, Lejla Gradjan, Julia Zajac, Kacper Gil, Yvonne Thokozile Nduku, Anna M. Blom   +6 more
wiley   +1 more source

National survey on pediatric respiratory physiotherapy units: primary ciliary dyskinesia and non-CF bronchiectasis

Italian Journal of Pediatrics
Background Currently, there is a lack of data concerning the organization and characteristics of Italian pediatric physiotherapy units for the treatment of patients with chronic lung diseases, especially those with rare conditions such as Primary Ciliary
Beatrice Tani, Nicola Ullmann, Paola Leone, Alessandra Boni, Eugenio Barbieri, Matteo D’Angelo, Sara De Dominicis, Beniamino Giacomodonato, Stefania Monduzzi, Irene Piermarini, Chiara Pizziconi, Beatrice Ferrari, Renato Cutrera   +12 more
doaj   +1 more source

Lung Cancer in Pulmonary Fibrosis: Tales of Epithelial Cell Plasticity [PDF]

, 2011
Lung epithelial cells exhibit a high degree of plasticity. Alterations to lung epithelial cell function are critically involved in several chronic lung diseases such as pulmonary fibrosis.
Adamson IY, Artinian V, Aubry MC, Bai JW, Baumgartner KB, Berschneider B, Brody AR, Chen PP, Chilosi M, Chilosi M, Coalson JJ, Corrin B, Dasari V, Dissanayake SK, Dobbs LG, Flozak AS, Fukuda Y, Ha HY, Haddad R, Herzog EL, Hironaka M, Hu J, Huang CL, Hubbard R, Huber MA, Jayachandran A, Kasai H, Kasper M, Kato N, Kawasaki H, Kawasaki H, Kim J, Kim K, Kim KK, Kuwano K, Königshoff M, Königshoff M, Königshoff M, Lee JM, Liu L, Maher TM, Margalit O, Mason RJ, Matthay MA, Mazieres J, Melanie Königshoff, Moon RT, Nagai A, Pan LH, Park J, Pennison M, Plataki M, Qunn L, Radisky DC, Rosas IO, Sato M, Selman M, Selman M, Soon LL, Sternlicht MD, Thannickal VJ, Thiery JP, Uematsu K, Willis BC, Willis BC, Willis BC, Zavadil J   +66 more
core   +1 more source

Single-Cell Transcriptomic Analysis of Human Lung Provides Insights into the Pathobiology of Pulmonary Fibrosis

American Journal of Respiratory and Critical Care Medicine, 2019
Rationale: The contributions of diverse cell populations in the human lung to pulmonary fibrosis pathogenesis are poorly understood. Single-cell RNA sequencing can reveal changes within individual cell populations during pulmonary fibrosis that are ...
P. Reyfman, J. Walter, N. Joshi, K. R. Anekalla, Alexandra McQuattie-Pimentel, Stephen Chiu, Ramiro Fernandez, Mahzad Akbarpour, Ching‐I Chen, Z. Ren, R. Verma, H. Abdala-Valencia, Kiwon Nam, Monica Chi, SeungHye Han, Francisco J. Gonzalez-Gonzalez, S. Soberanes, Satoshi Watanabe, Kinola J. N. Williams, A. S. Flozak, T. Nicholson, Vince K. Morgan, D. Winter, M. Hinchcliff, C. Hrusch, R. Guzy, C. Bonham, A. Sperling, R. Bag, R. Hamanaka, G. Mutlu, A. Yeldandi, Stacy A. Marshall, A. Shilatifard, L. Amaral, H. Perlman, J. Sznajder, A. Argento, C. Gillespie, J. Dematte, M. Jain, Benjamin D. Singer, K. Ridge, A. Lam, A. Bharat, S. Bhorade, C. Gottardi, G. S. Budinger, A. Misharin   +48 more
semanticscholar   +1 more source