Can continuous glucose monitoring predict cystic fibrosis-related diabetes and worse clinical outcome?
Jornal Brasileiro De Pneumologia : Publicacao Oficial Da Sociedade Brasileira De Pneumologia E Tisilogia
Zorron, Mariana M; Marson, Fernando Augusto Lima FAL; Morcillo, André Moreno AM; Gonçalves, Aline Cristina AC; El Beck, Mayra de Souza MS; Ribeiro, José Dirceu JD; Ribeiro, Antonio Fernando AF
Seamless Gene Correction in the Human Cystic Fibrosis Transmembrane Conductance Regulator Locus by Vector Replacement and Vector Insertion Events.
Frontiers In Genome Editing
Suzuki, Shingo S; Chosa, Keisuke K; Barillà, Cristina C; Yao, Michael M; Zuffardi, Orsetta O; Kai, Hirofumi H; Shuto, Tsuyoshi T; Suico, Mary Ann MA; Kan, Yuet W YW; Sargent, R Geoffrey RG; Gruenert, Dieter C DC
CFTR Modulation Reduces SARS-CoV-2 Infection in Human Bronchial Epithelial Cells.
Cells
Lotti, Virginia V; Merigo, Flavia F; Lagni, Anna A; Di Clemente, Andrea A; Ligozzi, Marco M; Bernardi, Paolo P; Rossini, Giada G; Concia, Ercole E; Plebani, Roberto R; Romano, Mario M; Sbarbati, Andrea A; Sorio, Claudio C; Gibellini, Davide D
Cilia Stimulatory and Antibacterial Activities of T2R Bitter Taste Receptor Agonist Diphenhydramine: Insights into Repurposing Bitter Drugs for Nasal Infections.
Pharmaceuticals (Basel, Switzerland)
Kuek, Li Eon LE; McMahon, Derek B DB; Ma, Ray Z RZ; Miller, Zoey A ZA; Jolivert, Jennifer F JF; Adappa, Nithin D ND; Palmer, James N JN; Lee, Robert J RJ
Elastic mucus strands impair mucociliary clearance in cystic fibrosis pigs.
Proceedings Of The National Academy Of Sciences Of The United States Of America
Pino-Argumedo, Maria I MI; Fischer, Anthony J AJ; Hilkin, Brieanna M BM; Gansemer, Nicholas D ND; Allen, Patrick D PD; Hoffman, Eric A EA; Stoltz, David A DA; Welsh, Michael J MJ; Abou Alaiwa, Mahmoud H MH
Risk Assessment for Patients with Chronic Respiratory Conditions in the Context of the SARS-CoV-2 Pandemic Statement of the German Respiratory Society with the Support of the German Association of Chest Physicians.
Respiration; International Review Of Thoracic Diseases
Lommatzsch, Marek M; Rabe, Klaus F KF; Taube, Christian C; Joest, Marcus M; Kreuter, Michael M; Wirtz, Hubert H; Blum, Torsten Gerriet TG; Kolditz, Martin M; Geerdes-Fenge, Hilte H; Otto-Knapp, Ralf R; Häcker, Brit B; Schaberg, Tom T; Ringshausen, Felix C FC; Vogelmeier, Claus F CF; Reinmuth, Niels N; Reck, Martin M; Gottlieb, Jens J; Konstantinides, Stavros S; Meyer, Joachim J; Worth, Heinrich H; Windisch, Wolfram W; Welte, Tobias T; Bauer, Torsten T
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
Physiological predictors of cardiorespiratory fitness in children and adolescents with cystic fibrosis without ventilatory limitation.
Therapeutic Advances In Respiratory Disease
Burghard, Marcella M; Takken, Tim T; Nap-van der Vlist, Merel M MM; Nijhof, Sanne L SL; van der Ent, C Kors CK; Heijerman, Harry G M HGM; Hulzebos, H J Erik HJE
Rothia mucilaginosa is an anti-inflammatory bacterium in the respiratory tract of patients with chronic lung disease.
The European Respiratory Journal
Rigauts, Charlotte C; Aizawa, Juliana J; Taylor, Steven S; Rogers, Geraint B GB; Govaerts, Matthias M; Cos, Paul P; Ostyn, Lisa L; Sims, Sarah S; Vandeplassche, Eva E; Sze, Mozes M; Dondelinger, Yves Y; Vereecke, Lars L; Van Acker, Heleen H; Simpson, Jodie L JL; Burr, Lucy L; Willems, Anne A; Tunney, Michael M MM; Cigana, Cristina C; Bragonzi, Alessandra A; Coenye, Tom T; Crabbé, Aurélie A
Identification and Characterization of Novel CFTR Potentiators.
Frontiers In Pharmacology
Gees, Maarten M; Musch, Sara S; Van der Plas, Steven S; Wesse, Anne-Sophie AS; Vandevelde, Ann A; Verdonck, Katleen K; Mammoliti, Oscar O; Hwang, Tzyh-Chang TC; Sonck, Kathleen K; Stouten, Pieter P; Swensen, Andrew M AM; Jans, Mia M; Van der Schueren, Jan J; Nelles, Luc L; Andrews, Martin M; Conrath, Katja K
Sweat rate analysis of ivacaftor potentiation of CFTR in non-CF adults.
Scientific Reports
Kim, Jeeyeon J; Farahmand, Miesha M; Dunn, Colleen C; Milla, Carlos E CE; Horii, Rina I RI; Thomas, Ewart A C EAC; Moss, Richard B RB; Wine, Jeffrey J JJ
Analysis of a large cohort of cystic fibrosis patients with severe liver disease indicates lung function decline does not significantly differ from that of the general cystic fibrosis population.
Plos One
Polineni, Deepika D; Piccorelli, Annalisa V AV; Hannah, William B WB; Dalrymple, Sarah N SN; Pace, Rhonda G RG; Durie, Peter R PR; Ling, Simon C SC; Knowles, Michael R MR; Stonebraker, Jaclyn R JR
Antibiotic multidrug resistance in the cystic fibrosis airway microbiome is associated with decreased diversity.
Heliyon
Hahn, Andrea A; Burrell, Aszia A; Fanous, Hani H; Chaney, Hollis H; Sami, Iman I; Perez, Geovanny F GF; Koumbourlis, Anastassios C AC; Freishtat, Robert J RJ; Crandall, Keith A KA
A novel triple combination of pharmacological chaperones improves F508del-CFTR correction.
Scientific Reports
Carlile, Graeme W GW; Yang, Qi Q; Matthes, Elizabeth E; Liao, Jie J; Radinovic, Stevo S; Miyamoto, Carol C; Robert, Renaud R; Hanrahan, John W JW; Thomas, David Y DY
Residual function of cystic fibrosis mutants predicts response to small molecule CFTR modulators.
Jci Insight
Han, Sangwoo T ST; Rab, Andras A; Pellicore, Matthew J MJ; Davis, Emily F EF; McCague, Allison F AF; Evans, Taylor A TA; Joynt, Anya T AT; Lu, Zhongzhou Z; Cai, Zhiwei Z; Raraigh, Karen S KS; Hong, Jeong S JS; Sheppard, David N DN; Sorscher, Eric J EJ; Cutting, Garry R GR
Molecular Mechanism of Action of Trimethylangelicin Derivatives as CFTR Modulators.
Frontiers In Pharmacology
Laselva, Onofrio O; Marzaro, Giovanni G; Vaccarin, Christian C; Lampronti, Ilaria I; Tamanini, Anna A; Lippi, Giuseppe G; Gambari, Roberto R; Cabrini, Giulio G; Bear, Christine E CE; Chilin, Adriana A; Dechecchi, Maria C MC
SLC6A14, an amino acid transporter, modifies the primary CF defect in fluid secretion.
Elife
Ahmadi, Saumel S; Xia, Sunny S; Wu, Yu-Sheng YS; Di Paola, Michelle M; Kissoon, Randolph R; Luk, Catherine C; Lin, Fan F; Du, Kai K; Rommens, Johanna J; Bear, Christine E CE
V232D mutation in patients with cystic fibrosis: Not so rare, not so mild.
Medicine
Fernández-Lorenzo, Ana E AE; Moreno-Álvarez, Ana A; Colon-Mejeras, Cristóbal C; Barros-Angueira, Francisco F; Solar-Boga, Alfonso A; Sirvent-Gómez, Josep J; Couce, María L ML; Leis, Rosaura R
Creating longitudinal datasets and cleaning existing data identifiers in a cystic fibrosis registry using a novel Bayesian probabilistic approach from astronomy.
Plos One
Hurley, Peter Donald PD; Oliver, Seb S; Mehta, Anil A
A G542X cystic fibrosis mouse model for examining nonsense mutation directed therapies.
Plos One
McHugh, Daniel R DR; Steele, Miarasa S MS; Valerio, Dana M DM; Miron, Alexander A; Mann, Rachel J RJ; LePage, David F DF; Conlon, Ronald A RA; Cotton, Calvin U CU; Drumm, Mitchell L ML; Hodges, Craig A CA
Cigarette Smoke-Induced Acquired Dysfunction of Cystic Fibrosis Transmembrane Conductance Regulator in the Pathogenesis of Chronic Obstructive Pulmonary Disease.
Oxidative Medicine And Cellular Longevity
Shi, Juan J; Li, Hui H; Yuan, Chao C; Luo, Meihui M; Wei, Jun J; Liu, Xiaoming X
Data from the US and UK cystic fibrosis registries support disease modification by CFTR modulation with ivacaftor.
Thorax
Bessonova, Leona L; Volkova, Nataliya N; Higgins, Mark M; Bengtsson, Leif L; Tian, Simon S; Simard, Christopher C; Konstan, Michael W MW; Sawicki, Gregory S GS; Sewall, Ase A; Nyangoma, Stephen S; Elbert, Alexander A; Marshall, Bruce C BC; Bilton, Diana D
Assembly and Functional Analysis of an S/MAR Based Episome with the Cystic Fibrosis Transmembrane Conductance Regulator Gene.
International Journal Of Molecular Sciences
De Rocco, Davide D; Pompili, Barbara B; Castellani, Stefano S; Morini, Elena E; Cavinato, Luca L; Cimino, Giuseppe G; Mariggiò, Maria A MA; Guarnieri, Simone S; Conese, Massimo M; Del Porto, Paola P; Ascenzioni, Fiorentina F
Improving imputation in disease-relevant regions: lessons from cystic fibrosis.
Npj Genomic Medicine
Panjwani, Naim N; Xiao, Bowei B; Xu, Lizhen L; Gong, Jiafen J; Keenan, Katherine K; Lin, Fan F; He, Gengming G; Baskurt, Zeynep Z; Kim, Sangook S; Zhang, Lin L; Esmaeili, Mohsen M; Blackman, Scott S; Scherer, Stephen W SW; Corvol, Harriet H; Drumm, Mitchell M; Knowles, Michael M; Cutting, Garry G; Rommens, Johanna M JM; Sun, Lei L; Strug, Lisa J LJ
Gap Junctions Are Involved in the Rescue of CFTR-Dependent Chloride Efflux by Amniotic Mesenchymal Stem Cells in Coculture with Cystic Fibrosis CFBE41o- Cells.
Stem Cells International
Carbone, Annalucia A; Zefferino, Roberto R; Beccia, Elisa E; Casavola, Valeria V; Castellani, Stefano S; Di Gioia, Sante S; Giannone, Valentina V; Seia, Manuela M; Angiolillo, Antonella A; Colombo, Carla C; Favia, Maria M; Conese, Massimo M
Fluctuations in airway bacterial communities associated with clinical states and disease stages in cystic fibrosis.
Plos One
Carmody, Lisa A LA; Caverly, Lindsay J LJ; Foster, Bridget K BK; Rogers, Mary A M MAM; Kalikin, Linda M LM; Simon, Richard H RH; VanDevanter, Donald R DR; LiPuma, John J JJ
Initial acquisition and succession of the cystic fibrosis lung microbiome is associated with disease progression in infants and preschool children.
Plos Pathogens
Muhlebach, Marianne S MS; Zorn, Bryan T BT; Esther, Charles R CR; Hatch, Joseph E JE; Murray, Conor P CP; Turkovic, Lidija L; Ranganathan, Sarath C SC; Boucher, Richard C RC; Stick, Stephen M SM; Wolfgang, Matthew C MC
Peripheral quantitative computed tomography detects differences at the radius in prepubertal children with cystic fibrosis compared to healthy controls.
Plos One
O'Brien, Catherine E CE; Com, Gulnur G; Fowlkes, John J; Tang, Xinyu X; James, Laura P LP
Up-to-date and projected estimates of survival for people with cystic fibrosis using baseline characteristics: A longitudinal study using UK patient registry data.
Journal Of Cystic Fibrosis : Official Journal Of The European Cystic Fibrosis Society
Keogh, Ruth H RH; Szczesniak, Rhonda R; Taylor-Robinson, David D; Bilton, Diana D
Conditionally reprogrammed primary airway epithelial cells maintain morphology, lineage and disease specific functional characteristics.
Scientific Reports
Martinovich, Kelly M KM; Iosifidis, Thomas T; Buckley, Alysia G AG; Looi, Kevin K; Ling, Kak-Ming KM; Sutanto, Erika N EN; Kicic-Starcevich, Elizabeth E; Garratt, Luke W LW; Shaw, Nicole C NC; Montgomery, Samuel S; Lannigan, Francis J FJ; Knight, Darryl A DA; Kicic, Anthony A; Stick, Stephen M SM
SLC6A14 Is a Genetic Modifier of Cystic Fibrosis That Regulates Pseudomonas aeruginosa Attachment to Human Bronchial Epithelial Cells.
Mbio
Di Paola, Michelle M; Park, Amber J AJ; Ahmadi, Saumel S; Roach, Elyse J EJ; Wu, Yu-Sheng YS; Struder-Kypke, Michaela M; Lam, Joseph S JS; Bear, Christine E CE; Khursigara, Cezar M CM
Relation of Ultrasound Findings and Abdominal Symptoms obtained with the CFAbd-Score in Cystic Fibrosis Patients.
Scientific Reports
Tabori, Harold H; Jaudszus, Anke A; Arnold, Christin C; Mentzel, Hans-Joachim HJ; Lorenz, Michael M; Michl, Ruth K RK; Lehmann, Thomas T; Renz, Diane M DM; Mainz, Jochen G JG