CAGE sequencing reveals CFTR-dependent dysregulation of type I IFN signaling in activated cystic fibrosis macrophages.
Science Advances
Gillan, Jonathan L JL; Chokshi, Mithil M; Hardisty, Gareth R GR; Clohisey Hendry, Sara S; Prasca-Chamorro, Daniel D; Robinson, Nicola J NJ; Lasota, Benjamin B; Clark, Richard R; Murphy, Lee L; Whyte, Moira K B MKB; Baillie, J Kenneth JK; Davidson, Donald J DJ; Bao, Gang G; Gray, Robert D RD
Critical role of down-regulated in adenoma bicarbonate transporter in linaclotide stimulated intestinal bicarbonate secretion.
Biorxiv : The Preprint Server For Biology
Sarthi, Jessica B JB; Trumbull, Annie M AM; Abazari, Shayda M SM; van Unen, Vincent V; Chan, Joshua E JE; Joo, Nam Soo NS; Jiang, Yanfen Y; Kuo, Calvin J CJ; Sellers, Zachary M ZM
Brazilian guidelines for the pharmacological treatment of the pulmonary symptoms of cystic fibrosis. Official document of the Sociedade Brasileira de Pneumologia e Tisiologia (SBPT, Brazilian Thoracic Association).
Jornal Brasileiro De Pneumologia : Publicacao Oficial Da Sociedade Brasileira De Pneumologia E Tisilogia
Athanazio, Rodrigo Abensur RA; Tanni, Suzana Erico SE; Ferreira, Juliana J; Dalcin, Paulo de Tarso Roth PTR; Fuccio, Marcelo B de MB; Esposito, Concetta C; Canan, Mariane Gonçalves Martynychen MGM; Coelho, Liana Sousa LS; Firmida, Mônica de Cássia MC; Almeida, Marina Buarque de MB; Marostica, Paulo José Cauduro PJC; Monte, Luciana de Freitas Velloso LFV; Souza, Edna Lúcia EL; Pinto, Leonardo Araujo LA; Rached, Samia Zahi SZ; Oliveira, Verônica Stasiak Bednarczuk de VSB; Riedi, Carlos Antonio CA; Silva Filho, Luiz Vicente Ribeiro Ferreira da LVRFD
Individualized approach to elexacaftor/tezacaftor/ivacaftor dosing in cystic fibrosis, in response to self-reported anxiety and neurocognitive adverse events: A case series.
Frontiers In Pharmacology
Ibrahim, Hisham H; Danish, Hammad H; Morrissey, David D; Deasy, Kevin F KF; McCarthy, Mairead M; Dorgan, James J; Fleming, Claire C; Howlett, Ciara C; Twohig, Sarah S; Vagg, Tamara T; Murphy, Desmond M DM; Maher, Michael M; Plant, Barry J BJ
ROCK STUDY in CF: sustained anti-inflammatory effects of lumacaftor-ivacaftor in sputum and peripheral blood samples of adult patients with cystic fibrosis-an observational study.
Bmj Open Respiratory Research
Arooj, Parniya P; Morrissy, David V DV; McCarthy, Yvonne Y; Vagg, Tamara T; McCarthy, Mairead M; Fleming, Claire C; Daly, Mary M; Eustace, Joseph A JA; Murphy, Desmond M DM; Plant, B J BJ
The Cystic Fibrosis Transmembrane Conductance Regulator Gene (CFTR) Is under Post-Transcriptional Control of microRNAs: Analysis of the Effects of agomiRNAs Mimicking miR-145-5p, miR-101-3p, and miR-335-5p.
Non-Coding Rna
Papi, Chiara C; Gasparello, Jessica J; Zurlo, Matteo M; Cosenza, Lucia Carmela LC; Gambari, Roberto R; Finotti, Alessia A
Long-term effects of lumacaftor/ivacaftor on paranasal sinus abnormalities in children with cystic fibrosis detected with magnetic resonance imaging.
Frontiers In Pharmacology
Wucherpfennig, Lena L; Wuennemann, Felix F; Eichinger, Monika M; Seitz, Angelika A; Baumann, Ingo I; Stahl, Mirjam M; Graeber, Simon Y SY; Zhao, Shengkai S; Chung, Jaehi J; Schenk, Jens-Peter JP; Alrajab, Abdulsattar A; Kauczor, Hans-Ulrich HU; Mall, Marcus A MA; Sommerburg, Olaf O; Wielpütz, Mark O MO
A novel thiol-saccharide mucolytic for the treatment of muco-obstructive lung diseases.
The European Respiratory Journal
Addante, Annalisa A; Raymond, Wilfred W; Gitlin, Irina I; Charbit, Annabelle A; Orain, Xavier X; Wolfe Scheffler, Aaron A; Kuppe, Aditi A; Duerr, Julia J; Daniltchenko, Maria M; Drescher, Marika M; Graeber, Simon Y SY; Healy, Anne-Marie AM; Oscarson, Stefan S; Fahy, John V JV; Mall, Marcus A MA
Pathogenesis of Achromobacter xylosoxidans respiratory infections: colonization and persistence of airway epithelia and differential gene expression in synthetic cystic fibrosis sputum medium.
Biorxiv : The Preprint Server For Biology
Billiot, Caitlin E CE; McDaniel, Melissa S MS; Swords, W Edward WE; Lindgren, Natalie R NR
Easy-to-Build and Reusable Microfluidic Device for the Dynamic Culture of Human Bronchial Cystic Fibrosis Epithelia.
Acs Biomaterials Science & Engineering
Mazio, Claudia C; Scognamiglio, Laura S LS; Passariello, Roberta R; Panzetta, Valeria V; Casale, Costantino C; Urciuolo, Francesco F; Galietta, Luis J V LJV; Imparato, Giorgia G; Netti, Paolo A PA
Immunoreactive Trypsinogen in Infants Born to Women with Cystic Fibrosis Taking Elexacaftor-Tezacaftor-Ivacaftor.
International Journal Of Neonatal Screening
Patel, Payal P; Yeley, Jana J; Brown, Cynthia C; Wesson, Melissa M; Lesko, Barbara G BG; Slaven, James E JE; Chmiel, James F JF; Jain, Raksha R; Sanders, Don B DB
Immunosuppression reduces rAAV2.5T neutralizing antibodies that limit efficacy following repeat dosing to ferret lungs.
Molecular Therapy. Methods & Clinical Development
Tang, Yinghua Y; Fakhari, Shahab S; Huntemann, Eric D ED; Feng, Zehua Z; Wu, Peipei P; Feng, William Y WY; Lei, Junying J; Yuan, Feng F; Excoffon, Katherine J KJ; Wang, Kai K; Limberis, Maria P MP; Kolbeck, Roland R; Yan, Ziying Z; Engelhardt, John F JF
Association of Oxygen Therapy with the Natural Disease Progression of Cystic Fibrosis: A Multi-State Model of the European Cystic Fibrosis Society Patient Registry.