Rowe SM, Miller S, Sorscher EJ. Cystic fibrosis. N Engl J Med. 2005;352:1992–2001.

Article  CAS  PubMed  Google Scholar 

Cystic Fibrosis Mutation Database. [cited 2023 Apr 1]. http://www.genet.sickkids.on.ca/

Cystic Fibrosis Foundation Patient Registry. 2021 Annual Data Report. 2022.

Orenti A, Zolin A, Jung A, van Rens J et al. ECFS Patient Registry Annual Report 2020. 2022.

Cheng SH, Gregory RJ, Marshall J, Paul S, Souza DW, White GA, et al. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis. Cell. 1990;63:827–34.

Article  CAS  PubMed  Google Scholar 

Denning GM, Ostedgaard LS, Welsh MJ. Abnormal localization of cystic fibrosis transmembrane conductance regulator in primary cultures of cystic fibrosis airway epithelia. J Cell Biol. 1992;118:551–9.

Article  CAS  PubMed  Google Scholar 

Welsh MJ, Smith AE. Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis. Cell. 1993;73:1251–4.

Article  CAS  PubMed  Google Scholar 

Dalemans W, Barbry P, Champigny G, Jallat S, Jallat S, Dott K, et al. Altered chloride ion channel kinetics associated with the ∆F508 cystic fibrosis mutation. Nature. 1991;354:526–8.

Article  CAS  PubMed  Google Scholar 

Lukacs GL, Chang XB, Bear C, Kartner N, Mohamed A, Riordan JR, et al. The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells. J Biol Chem. 1993;268:21592–8.

Article  CAS  PubMed  Google Scholar 

Okiyoneda T, Barrière H, Bagdány M, Rabeh WM, Du K, Höhfeld J, et al. Peripheral protein quality control removes unfolded CFTR from the plasma membrane. Science. 2010;329:805–10.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Liu X, Dawson DC. Cystic fibrosis transmembrane Conductance Regulator (CFTR) potentiators protect G551D but not ∆F508 CFTR from Thermal instability. Biochemistry. 2014;53:5613–8.

Article  CAS  PubMed  Google Scholar 

Wang W, Okeyo GO, Tao B, Hong JS, Kirk KL. Thermally unstable gating of the most common cystic fibrosis Mutant Channel (∆F508). J Biol Chem. 2011;286:41937–48.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Veit G, Avramescu RG, Chiang AN, Houck SA, Cai Z, Peters KW, et al. From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations. Mol Biol Cell. 2016;27:424–33.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Okiyoneda T, Veit G, Dekkers JF, Bagdany M, Soya N, Xu H, et al. Mechanism-based corrector combination restores ∆F508-CFTR folding and function. Nat Chem Biol. 2013;9:444–54.

Article  CAS  PubMed  Google Scholar 

Veit G, Roldan A, Hancock MA, Fonte DFD, Xu H, Hussein M et al. Allosteric folding correction of F508del and rare CFTR mutants by elexacaftor-tezacaftor-ivacaftor (Trikafta) combination. JCI Insight. 2020 [cited 2023 Apr 1];5. https://insight.jci.org/articles/view/139983

Laselva O, Bartlett C, Gunawardena TNA, Ouyang H, Eckford PDW, Moraes TJ et al. Rescue of multiple class II CFTR mutations by elexacaftor + tezacaftor + ivacaftor mediated in part by the dual activities of elexacaftor as both corrector and potentiator. European Respiratory Journal. 2021 [cited 2023 Apr 1];57. https://erj.ersjournals.com/content/57/6/2002774

Bihler H, Sivachenko A, Millen L, Bhatt P, Patel AT, Chin J, et al. In vitro modulator responsiveness of 655 CFTR variants found in people with cystic fibrosis. J Cyst Fibros. 2024;23:664–75.

Article  CAS  PubMed  Google Scholar 

Aspinall SA, Mackintosh KA, Hill DM, Cope B, McNarry MA. Evaluating the Effect of Kaftrio on perspectives of Health and Wellbeing in individuals with cystic fibrosis. Int J Environ Res Public Health. 2022;19:6114.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Tabary O, Boncoeur E, de Martin R, Pepperkok R, Clément A, Schultz C, et al. Calcium-dependent regulation of NF-κB activation in cystic fibrosis airway epithelial cells. Cell Signal. 2006;18:652–60.

Article  CAS  PubMed  Google Scholar 

Antigny F, Norez C, Becq F, Vandebrouck C. Calcium homeostasis is abnormal in cystic fibrosis airway epithelial cells but is normalized after rescue of F508del-CFTR. Cell Calcium. 2008;43:175–83.

Article  CAS  PubMed  Google Scholar 

Ribeiro CMP, Paradiso AM, Carew MA, Shears SB, Boucher RC. Cystic fibrosis Airway Epithelial Ca2 + i signaling: THE MECHANISM FOR THE LARGER AGONIST-MEDIATED Ca2 + i SIGNALS IN HUMAN CYSTIC FIBROSIS AIRWAY EPITHELIA *. J Biol Chem. 2005;280:10202–9.

Article  CAS  PubMed  Google Scholar 

Ribeiro CMP, Paradiso AM, Schwab U, Perez-Vilar J, Jones L, O’Neal W, et al. Chronic Airway Infection/Inflammation induces a Ca2 + i-dependent Hyperinflammatory response in human cystic fibrosis Airway Epithelia *. J Biol Chem. 2005;280:17798–806.

Article  CAS  PubMed  Google Scholar 

Ribeiro CMP. The role of intracellular calcium signals in inflammatory responses of polarised cystic Fibrosis Human Airway Epithelia. Drugs R D. 2006;7:17–31.

Article  CAS  PubMed  Google Scholar 

Martins JR, Kongsuphol P, Sammels E, Dahimène S, AlDehni F, Clarke LA, et al. F508del-CFTR increases intracellular Ca2+ signaling that causes enhanced calcium-dependent Cl– conductance in cystic fibrosis. Biochimica et Biophysica Acta (BBA) -. Mol Basis Disease. 2011;1812:1385–92.

Article  CAS  Google Scholar 

Vachel L, Norez C, Becq F, Vandebrouck C. Effect of VX-770 (Ivacaftor) and OAG on Ca2 + influx and CFTR activity in G551D and F508del-CFTR expressing cells. J Cyst Fibros. 2013;12:584–91.

Article  CAS  PubMed  Google Scholar 

Balghi H, Robert R, Rappaz B, Zhang X, Wohlhuter-Haddad A, Evagelidis A, et al. Enhanced Ca2 + entry due to Orai1 plasma membrane insertion increases IL-8 secretion by cystic fibrosis airways. FASEB J. 2011;25:4274–91.

Article  CAS  PubMed  PubMed Central  Google Scholar 

Philippe R, Antigny F, Buscaglia P, Norez C, Becq F, Frieden M, et al. SERCA and PMCA pumps contribute to the deregulation of Ca2 + homeostasis in human CF epithelial cells. Biochimica et Biophysica Acta (BBA) -. Mol Cell Res. 2015;1853:892–903.

CAS  Google Scholar 

Grebert C, Becq F, Vandebrouck C. Focus on TRP channels in cystic fibrosis. Cell Calcium. 2019;81:29–37.

Article  CAS  PubMed  Google Scholar 

Rimessi A, Vitto VAM, Patergnani S, Pinton P. Update on Calcium Signaling in Cystic Fibrosis Lung Disease. Frontiers in Pharmacology. 2021 [cited 2023 Apr 1];12. https://www.frontiersin.org/articles/

Egan ME, Glöckner-Pagel J, Ambrose CA, Cahill PA, Pappoe L, Balamuth N, et al. Calcium-pump inhibitors induce functional surface expression of ∆F508-CFTR protein in cystic fibrosis epithelial cells. Nat Med. 2002;8:485–92.

Article  CAS  PubMed  Google Scholar 

Egan ME, Pearson M, Weiner SA, Rajendran V, Rubin D, Glöckner-Pagel J, et al. Curcumin, a Major Constituent of Turmeric, corrects cystic fibrosis defects. Science. 2004;304:600–2.

Article  CAS  PubMed  Google Scholar 

Norez C, Antigny F, Becq F, Vandebrouck C. Maintaining low Ca2 + level in the endoplasmic reticulum restores abnormal endogenous F508del-CFTR trafficking in Airway Epithelial cells. Traffic. 2006;7:562–73.

Article  CAS  PubMed  Google Scholar 

Antigny F, Girardin N, Raveau D, Frieden M, Becq F, Vandebrouck C. Dysfunction of mitochondria Ca2 + uptake in cystic fibrosis airway epithelial cells. Mitochondrion. 2009;9:232–41.

Article  CAS  PubMed  Google Scholar 

Shapiro BL, Lam LF. Intracellular calcium in cystic fibrosis heterozygotes. Life Sci. 1987;40:2361–6.

Article  CAS  PubMed  Google Scholar 

Bargon J, Trapnell BC, Chu CS, Rosenthal ER, Yoshimura K, Guggino WB, et al. Down-regulation of cystic fibrosis transmembrane conductance regulator gene expression by agents that modulate intracellular divalent cations. Mol Cell Biol. 1992;12:1872–8.