Results 51 to 60 of about 10,277 (199)

Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity. [PDF]

, 2019
Deletion of phenylalanine 508 (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel is the most frequent mutation causing cystic fibrosis (CF). F508del-CFTR is misfolded and prematurely degraded. Recently thymosin a-1 (
Armirotti, Andrea, Braccia, Clarissa, Cholon, Deborah M, Galietta, Luis JV, Gentzsch, Martina, Guidone, Daniela, Hanrahan, John W, Lukacs, Gergely L, Matthes, Elizabeth, Pedemonte, Nicoletta, Phuan, Puay-Wah, Tomati, Valeria, Veit, Guido, Verkman, Alan S   +13 more
core  

Newborn screening for cystic fibrosis [PDF]

, 2016
Since the late 1970s when the potential of the immunoreactive trypsinogen assay for early identification of infants with cystic fibrosis was first recognised, the performance of newborn blood spot screening (NBS) has been continually assessed and its use
Castellani, C, Massie, J, Sontag, M, Southern, KW   +3 more
core   +1 more source

Soft Artificial Ciliary Brush with Integrated Haptic Feedback for Efficient Airway Mucus Cleaning

Advanced Intelligent Systems, EarlyView.
A fundamental mucus cleaning mechanism is reported, which utilizes both artificial cilia mechanical sweeping and suction‐based mucus cleaning. A novel haptic feedback interface for the artificial cilia provides touch feedback on tissue contact, enhancing operator control and safety.
Zhongming Lyu, Yusheng Wang, Ruijian Ge, Darren Wang, Matthew Bacchetta, Caitlin T. Demarest, Fabien Maldonado, Xiaoguang Dong   +7 more
wiley   +1 more source

Nanomolar-potency 'co-potentiator' therapy for cystic fibrosis caused by a defined subset of minimal function CFTR mutants. [PDF]

, 2019
Available CFTR modulators provide no therapeutic benefit for cystic fibrosis (CF) caused by many loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) chloride channel, including N1303K. We previously introduced the
Finkbeiner, Walter E, Haggie, Peter M, Nielson, Dennis W, Phuan, Puay-Wah, Rivera, Amber A, Tan, Joseph-Anthony, Verkman, Alan S, Zlock, Lorna   +7 more
core  

Phagocytosis depends on TRPV2-mediated calcium influx and requires TRPV2 in lipids rafts: alteration in macrophages from patients with cystic fibrosis. [PDF]

, 2018
Whereas many phagocytosis steps involve ionic fluxes, the underlying ion channels remain poorly defined. As reported in mice, the calcium conducting TRPV2 channel impacts the phagocytic process.
A Abu-Arish, A Aderem, A Di, A Magenau, A Oláh, A Perez, A Perálvarez-Marín, B Svobodova, D Wang, DJ Hackam, E Hisanaga, E Pernet, F Antigny, F Antigny, G Santoni, H Balghi, H Grassmé, H Matsui, JD Young, JH Guo, Jvan Grevenynghe, K Abe, K Aoyagi, K Simonin-Le Jeune, K-Y Jih, L Ambrus, L Nie, L Petrocellis De, L Wei, LM Carrithers, LV Deriy, M Lévêque, M Nabissi, M Nagasawa, M Nagasawa, M Saito, MD Carrithers, MJ Caterina, MP Kowalski, N Qin, NE Alexis, P Schatzlmaier, P Zhang, T Hishikawa, T Ma, TM Link, Y Iwata, É Sághy   +47 more
core   +4 more sources

Uncovering Cystic Fibrosis Carrier: Insights From a Heterozygous CFTR‐F508del Rabbit Model

International Forum of Allergy &Rhinology, EarlyView.
ABSTRACT Background Chronic rhinosinusitis (CRS) is a heterogeneous inflammatory disorder frequently associated with impaired mucociliary clearance and bacterial infection. Individuals carrying a single cystic fibrosis transmembrane conductance regulator (CFTR) mutation exhibit partial CFTR dysfunction and are increasingly recognized as being at risk ...
Do‐Yeon Cho, Alexis E. McFeely, Daniel Skinner, Shaoyan Zhang, Justin Hedvat, Weslee Ping‐ay, Daniel M. Beswick, Justin H. Turner, Bradford A. Woodworth, Jie Xu   +9 more
wiley   +1 more source

Q1291H-CFTR molecular dynamics simulations and ex vivo theratyping in nasal epithelial models and clinical response to elexacaftor/tezacaftor/ivacaftor in a Q1291H/F508del patient

Frontiers in Molecular Biosciences, 2023
Background: Cystic fibrosis (CF) is caused by a wide spectrum of mutations in the CF transmembrane conductance regulator (CFTR) gene, with some leading to non-classical clinical presentations.
Katelin M. Allan, Katelin M. Allan, Katelin M. Allan, Miro A. Astore, Egi Kardia, Egi Kardia, Egi Kardia, Sharon L. Wong, Sharon L. Wong, Sharon L. Wong, Laura K. Fawcett, Laura K. Fawcett, Laura K. Fawcett, Laura K. Fawcett, Jessica L. Bell, Jessica L. Bell, Simone Visser, Po-Chia Chen, Renate Griffith, Adam Jaffe, Adam Jaffe, Adam Jaffe, Sheila Sivam, Orazio Vittorio, Orazio Vittorio, Serdar Kuyucak, Shafagh A. Waters, Shafagh A. Waters, Shafagh A. Waters, Shafagh A. Waters   +29 more
doaj   +1 more source

Determining the Minimal Clinically Important Difference of the 40‐Item Smell Identification Test in People With Cystic Fibrosis

International Forum of Allergy &Rhinology, EarlyView.
ABSTRACT Background Chronic rhinosinusitis (CRS) and olfactory dysfunction (OD) are highly prevalent among people with cystic fibrosis (PwCF) and negatively impact quality of life. The 40‐item Smell Identification Test (SIT) is widely used to assess psychophysical olfaction, but a CF‐specific minimal clinically important difference (MCID) has not been ...
Eugene Oh, Jessa E. Miller, Michelle J. Lee, Anna Zemke, David Baraghoshi, Matthew J. Strand, Jeremiah A. Alt, Todd E. Bodner, Michael Chang, Naweed I. Chowdhury, Patricia H. Eshaghian, Anne E. Getz, Jennifer L. Goralski, David A. Gudis, Peter H. Hwang, Ashoke Khanwalkar, Adam J. Kimple, Jivianne T. Lee, Douglas A. Li, Jess C. Mace, Jayakar V. Nayak, Jonathan B. Overdevest, Zara Patel, Daniella K. Safatian, Rodney J. Schlosser, Brent Senior, Amanda L. Stapleton, Timothy L. Smith, Zachary M. Soler, Jeffrey D. Suh, Grant A. Turner, Marilene B. Wang, Milene T. Saavedra, Jennifer L. Taylor‐Cousar, Daniel M. Beswick   +34 more
wiley   +1 more source

Effects of CFTR Modulators on Pseudomonas aeruginosa Infections in Cystic Fibrosis

Infectious Disease Reports
Background: Cystic fibrosis (CF) is an autosomal recessive disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
Camelia Corina Pescaru, Alexandru Florian Crișan, Adelina Marițescu, Vlad Cărunta, Monica Marc, Ștefan Dumitrache-Rujinski, Sorina Laitin, Cristian Oancea   +7 more
doaj   +1 more source

Bronchiectasis:a case-based approach to investigation and management [PDF]

, 2018
Bronchiectasis is a chronic respiratory disease characterised by a syndrome of productive cough and recurrent respiratory infections due to permanent dilatation of the bronchi.
Agarwal, Agarwal, Bonaiti, Cartier, Chu, Cole, Erasmus, Farrell, Greenberger, Gremmo, Hosie, James D. Chalmers, Martina Contarini, Mirsaeidi, Neeld, Nicolini, Shapiro, Simon Finch   +17 more
core   +3 more sources