Treatment With LAU-7b Complements CFTR Modulator Therapy by Improving Lung Physiology and Normalizing Lipid Imbalance Associated With CF Lung Disease.
Frontiers In Pharmacology
Centorame, Amanda A; Dumut, Daciana Catalina DC; Youssef, Mina M; Ondra, Martin M; Kianicka, Irenej I; Shah, Juhi J; Paun, Radu Alexandru RA; Ozdian, Tomas T; Hanrahan, John W JW; Gusev, Ekaterina E; Petrof, Basil B; Hajduch, Marian M; Pislariu, Radu R; De Sanctis, Juan Bautista JB; Radzioch, Danuta D
Translational Research in Cystic Fibrosis: From Bench to Beside.
Frontiers In Pediatrics
Garcia, Laura de Castro E LCE; Petry, Lucas Montiel LM; Germani, Pedro Augusto Van Der Sand PAVS; Xavier, Luiza Fernandes LF; de Barros, Paula Barros PB; Meneses, Amanda da Silva ADS; Prestes, Laura Menestrino LM; Bittencourt, Luana Braga LB; Pieta, Marina Puerari MP; Friedrich, Frederico F; Pinto, Leonardo Araújo LA
Accounting for population structure in genetic studies of cystic fibrosis.
Hgg Advances
Kingston, Hanley H; Stilp, Adrienne M AM; Gordon, William W; Broome, Jai J; Gogarten, Stephanie M SM; Ling, Hua H; Barnard, John J; Dugan-Perez, Shannon S; Ellinor, Patrick T PT; Gabriel, Stacey S; Germer, Soren S; Gibbs, Richard A RA; Gupta, Namrata N; Rice, Kenneth K; Smith, Albert V AV; Zody, Michael C MC; , ; , ; Blackman, Scott M SM; Cutting, Garry G; Knowles, Michael R MR; Zhou, Yi-Hui YH; Rosenfeld, Margaret M; Gibson, Ronald L RL; Bamshad, Michael M; Fohner, Alison A; Blue, Elizabeth E EE
Short-Term Effects of Elexacaftor/Tezacaftor/Ivacaftor Combination on Glucose Tolerance in Young People With Cystic Fibrosis-An Observational Pilot Study.
Frontiers In Pediatrics
Korten, Insa I; Kieninger, Elisabeth E; Krueger, Linn L; Bullo, Marina M; Flück, Christa E CE; Latzin, Philipp P; Casaulta, Carmen C; Boettcher, Claudia C
Novel CFTR modulator combinations maximise rescue of G85E and N1303K in rectal organoids.
Erj Open Research
Ensinck, Marjolein M MM; De Keersmaecker, Liesbeth L; Ramalho, Anabela S AS; Cuyx, Senne S; Van Biervliet, Stephanie S; Dupont, Lieven L; Christ, Frauke F; Debyser, Zeger Z; Vermeulen, François F; Carlon, Marianne S MS
Efficacy and Safety of Triple Combination Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Patients With Cystic Fibrosis: A Meta-Analysis of Randomized Controlled Trials.
Frontiers In Pharmacology
Wang, Yizi Y; Ma, Bin B; Li, Wenya W; Li, Peiwen P
CFTR bearing variant p.Phe312del exhibits function inconsistent with phenotype and negligible response to ivacaftor.
Jci Insight
Raraigh, Karen S KS; Paul, Kathleen C KC; Goralski, Jennifer L JL; Worthington, Erin N EN; Faino, Anna V AV; Sciortino, Stanley S; Wang, Yiting Y; Aksit, Melis A MA; Ling, Hua H; Osorio, Derek L DL; Onchiri, Frankline M FM; Patel, Shivani U SU; Merlo, Christian A CA; Montemayor, Kristina K; Gibson, Ronald L RL; West, Natalie E NE; Thakerar, Amita A; Bridges, Robert J RJ; Sheppard, David N DN; Sharma, Neeraj N; Cutting, Garry R GR
The NSAID glafenine rescues class 2 CFTR mutants via cyclooxygenase 2 inhibition of the arachidonic acid pathway.
Scientific Reports
Carlile, Graeme W GW; Yang, Qi Q; Matthes, Elizabeth E; Liao, Jie J; Birault, Véronique V; Sneddon, Helen F HF; Poole, Darren L DL; Hall, Callum J CJ; Hanrahan, John W JW; Thomas, David Y DY
CFTR Modulators Restore Acidification of Autophago-Lysosomes and Bacterial Clearance in Cystic Fibrosis Macrophages.
Frontiers In Cellular And Infection Microbiology
Badr, Asmaa A; Eltobgy, Mostafa M; Krause, Kathrin K; Hamilton, Kaitlin K; Estfanous, Shady S; Daily, Kylene P KP; Abu Khweek, Arwa A; Hegazi, Ahmad A; Anne, Midhun N K MNK; Carafice, Cierra C; Robledo-Avila, Frank F; Saqr, Youssra Y; Zhang, Xiaoli X; Bonfield, Tracey L TL; Gavrilin, Mikhail A MA; Partida-Sanchez, Santiago S; Seveau, Stephanie S; Cormet-Boyaka, Estelle E; Amer, Amal O AO
Factors Predisposing the Response to Lumacaftor/Ivacaftor in People with Cystic Fibrosis.
Journal Of Personalized Medicine
Mésinèle, Julie J; Ruffin, Manon M; Guillot, Loïc L; Boëlle, Pierre-Yves PY; Corvol, Harriet H; On Behalf Of The French Cf Modifier Gene Study Investigators,
Elexacaftor/Tezacaftor/Ivacaftor in Patients with Cystic Fibrosis Homozygous for the F508del Mutation and Advanced Lung Disease: A 48-Week Observational Study.
Journal Of Clinical Medicine
Carnovale, Vincenzo V; Iacotucci, Paola P; Terlizzi, Vito V; Colangelo, Carmela C; Ferrillo, Lorenza L; Pepe, Angela A; Francalanci, Michela M; Taccetti, Giovanni G; Buonaurio, Serena S; Celardo, Assunta A; Salvadori, Laura L; Marsicovetere, Giovanni G; D'Andria, Michele M; Ferrara, Nicola N; Salvatore, Donatello D
Whole-genome analysis of human embryonic stem cells enables rational line selection based on genetic variation.
Cell Stem Cell
Merkle, Florian T FT; Ghosh, Sulagna S; Genovese, Giulio G; Handsaker, Robert E RE; Kashin, Seva S; Meyer, Daniel D; Karczewski, Konrad J KJ; O'Dushlaine, Colm C; Pato, Carlos C; Pato, Michele M; MacArthur, Daniel G DG; McCarroll, Steven A SA; Eggan, Kevin K
Publication Date: 2022-02-15
Variant appearance in text: CFTR: 1652G>A; Gly551Asp; rs75527207
Intestinal Inflammation and Alterations in the Gut Microbiota in Cystic Fibrosis: A Review of the Current Evidence, Pathophysiology and Future Directions.
Journal Of Clinical Medicine
Tam, Rachel Y RY; van Dorst, Josie M JM; McKay, Isabelle I; Coffey, Michael M; Ooi, Chee Y CY
REAL-world clinical effectiveness of ivacaftor therapy in the first 24 months in two infants with cystic fibrosis and different gating mutations-A case report.
Clinical Case Reports
Fuchs, Teresa T; Appelt, Dorothea D; Niedermayr, Katharina K; Ellemunter, Helmut H
Implementing the use of objective medication adherence data in routine clinical practice via the digital CFHealthHub platform: situation analysis and strategy development using the theoretical domains framework.
Implementation Science Communications
Girling, Carla C; Packham, Anna A; Robinson, Louisa L; Arden, Madelynne A MA; Hind, Daniel D; Wildman, Martin J MJ
A medium composition containing normal resting glucose that supports differentiation of primary human airway cells.
Scientific Reports
Morgan, Rachel R; Manfredi, Candela C; Easley, Kristen F KF; Watkins, Lionel D LD; Hunt, William R WR; Goudy, Steven L SL; Sorscher, Eric J EJ; Koval, Michael M; Molina, Samuel A SA
Molecular dynamics and functional characterization of I37R-CFTR lasso mutation provide insights into channel gating activity.
Iscience
Wong, Sharon L SL; Awatade, Nikhil T NT; Astore, Miro A MA; Allan, Katelin M KM; Carnell, Michael J MJ; Slapetova, Iveta I; Chen, Po-Chia PC; Capraro, Alexander A; Fawcett, Laura K LK; Whan, Renee M RM; Griffith, Renate R; Ooi, Chee Y CY; Kuyucak, Serdar S; Jaffe, Adam A; Waters, Shafagh A SA
Treatment of Polarized Cystic Fibrosis Airway Cells With HGF Prevents VX-661-Rescued F508del-CFTR Destabilization Caused by Prolonged Co-exposure to VX-770.
Esc peptides as novel potentiators of defective cystic fibrosis transmembrane conductance regulator: an unprecedented property of antimicrobial peptides.
Cellular And Molecular Life Sciences : Cmls
Ferrera, Loretta L; Cappiello, Floriana F; Loffredo, Maria Rosa MR; Puglisi, Elena E; Casciaro, Bruno B; Botta, Bruno B; Galietta, Luis J V LJV; Mori, Mattia M; Mangoni, Maria Luisa ML