» Articles » PMID: 33304402

The Era of CFTR Modulators: Improvements Made and Remaining Challenges

Overview
Journal Breathe (Sheff)
Specialty Pulmonary Medicine
Date 2020 Dec 11
PMID 33304402
Citations 19
Authors
Affiliations
Soon will be listed here.
Abstract

https://bit.ly/3aRafQF.

Citing Articles

Inhibiting CFTR through inh-172 in primary neutrophils reveals CFTR-specific functional defects.

Da Silva Cunha A, Blanter M, Renders J, Gouwy M, Lorent N, Boon M Sci Rep. 2024; 14(1):31237.

PMID: 39732786 PMC: 11682091. DOI: 10.1038/s41598-024-82535-z.


Identification of novel natural compounds against CFTR p.Gly628Arg pathogenic variant.

Khan M, Sakhawat A, Rehman R, Wali A, Ghani M, Akram A AMB Express. 2024; 14(1):99.

PMID: 39249658 PMC: 11383896. DOI: 10.1186/s13568-024-01762-9.


in chronic lung disease: untangling the dysregulated host immune response.

Nickerson R, Thornton C, Johnston B, Lee A, Cheng Z Front Immunol. 2024; 15:1405376.

PMID: 39015565 PMC: 11250099. DOI: 10.3389/fimmu.2024.1405376.


Cystic fibrosis management in pediatric population-from clinical features to personalized therapy.

Azoicai A, Lupu A, Trandafir L, Alexoae M, Alecsa M, Starcea I Front Pediatr. 2024; 12:1393193.

PMID: 38798310 PMC: 11116730. DOI: 10.3389/fped.2024.1393193.


Olive Leaf Extract (OLE) as a Novel Antioxidant That Ameliorates the Inflammatory Response in Cystic Fibrosis.

Allegretta C, Difonzo G, Caponio F, Tamma G, Laselva O Cells. 2023; 12(13).

PMID: 37443798 PMC: 10340374. DOI: 10.3390/cells12131764.


References
1.
Valley H, Bukis K, Bell A, Cheng Y, Wong E, Jordan N . Isogenic cell models of cystic fibrosis-causing variants in natively expressing pulmonary epithelial cells. J Cyst Fibros. 2018; 18(4):476-483. DOI: 10.1016/j.jcf.2018.12.001. View

2.
Paranjapye A, Ruffin M, Harris A, Corvol H . Genetic variation in CFTR and modifier loci may modulate cystic fibrosis disease severity. J Cyst Fibros. 2019; 19 Suppl 1:S10-S14. PMC: 7036019. DOI: 10.1016/j.jcf.2019.11.001. View

3.
Sagel S, Wagner B, Ziady A, Kelley T, Clancy J, Narvaez-Rivas M . Utilizing centralized biorepository samples for biomarkers of cystic fibrosis lung disease severity. J Cyst Fibros. 2019; 19(4):632-640. PMC: 7305052. DOI: 10.1016/j.jcf.2019.12.007. View

4.
Nick J, St Clair C, Jones M, Lan L, Higgins M . Ivacaftor in cystic fibrosis with residual function: Lung function results from an N-of-1 study. J Cyst Fibros. 2019; 19(1):91-98. DOI: 10.1016/j.jcf.2019.09.013. View

5.
Kelly A, De Leon D, Sheikh S, Camburn D, Kubrak C, Peleckis A . Islet Hormone and Incretin Secretion in Cystic Fibrosis after Four Months of Ivacaftor Therapy. Am J Respir Crit Care Med. 2018; 199(3):342-351. PMC: 6835076. DOI: 10.1164/rccm.201806-1018OC. View