Update in Cystic Fibrosis 2005

Overview

Journal Am J Respir Crit Care Med

Specialty Critical Care

Date 2006 Apr 25

PMID 16632633

Citations 11

Authors

Frank J Accurso

Affiliations

Soon will be listed here.

Citing Articles

Effect of CFTR Modulators on Oxidative Stress and Autophagy in Non-CFTR-Expressing Cells.

Scialo F, Cernera G, Polise L, Castaldo G, Amato F, Villella V Int J Mol Sci. 2024; 25(19).

PMID: 39408688 PMC: 11476568. DOI: 10.3390/ijms251910360.

Advanced lung organoids for respiratory system and pulmonary disease modeling.

Joo H, Min S, Cho S J Tissue Eng. 2024; 15:20417314241232502.

PMID: 38406820 PMC: 10894554. DOI: 10.1177/20417314241232502.

Phenotypic and Genetic Characterization of Carbapenemase and ESBLs Producing Gram-negative Bacteria (GNB) Isolated from Patients with Cystic Fibrosis (CF) in Tehran Hospitals.

Vali P, Shahcheraghi F, Seyfipour M, Zamani M, Allahyar M, Feizabadi M J Clin Diagn Res. 2014; 8(1):26-30.

PMID: 24596716 PMC: 3939568. DOI: 10.7860/JCDR/2014/6877.3916.

Disease-relevant proteostasis regulation of cystic fibrosis transmembrane conductance regulator.

Villella V, Esposito S, Bruscia E, Vicinanza M, Cenci S, Guido S Cell Death Differ. 2013; 20(8):1101-15.

PMID: 23686137 PMC: 3705602. DOI: 10.1038/cdd.2013.46.

Targeting autophagy as a novel strategy for facilitating the therapeutic action of potentiators on ΔF508 cystic fibrosis transmembrane conductance regulator.

Luciani A, Villella V, Esposito S, Gavina M, Russo I, Silano M Autophagy. 2012; 8(11):1657-72.

PMID: 22874563 PMC: 3494594. DOI: 10.4161/auto.21483.

References

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Baker S, Borowitz D, Duffy L, FitzPatrick L, Gyamfi J, Baker R . Pancreatic enzyme therapy and clinical outcomes in patients with cystic fibrosis. J Pediatr. 2005; 146(2):189-93. DOI: 10.1016/j.jpeds.2004.09.003. View

Voynow J, Fischer B, Roberts B, Proia A . Basal-like cells constitute the proliferating cell population in cystic fibrosis airways. Am J Respir Crit Care Med. 2005; 172(8):1013-8. DOI: 10.1164/rccm.200410-1398OC. View

Grasemann H, Schwiertz R, Matthiesen S, Racke K, Ratjen F . Increased arginase activity in cystic fibrosis airways. Am J Respir Crit Care Med. 2005; 172(12):1523-8. DOI: 10.1164/rccm.200502-253OC. View

Sloane A, Lindner R, Prasad S, Sebastian L, Pedersen S, Robinson M . Proteomic analysis of sputum from adults and children with cystic fibrosis and from control subjects. Am J Respir Crit Care Med. 2005; 172(11):1416-26. DOI: 10.1164/rccm.200409-1215OC. View

Davis P . Cystic fibrosis since 1938. Am J Respir Crit Care Med. 2005; 173(5):475-82. DOI: 10.1164/rccm.200505-840OE. View

de Jong P, Nakano Y, Hop W, Long F, Coxson H, Pare P . Changes in airway dimensions on computed tomography scans of children with cystic fibrosis. Am J Respir Crit Care Med. 2005; 172(2):218-24. DOI: 10.1164/rccm.200410-1311OC. View

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Sermet-Gaudelus I, Dechaux M, Vallee B, Fajac A, Girodon E, Nguyen-Khoa T . Chloride transport in nasal ciliated cells of cystic fibrosis heterozygotes. Am J Respir Crit Care Med. 2005; 171(9):1026-31. DOI: 10.1164/rccm.200406-740OC. View

11.

Brody A, Sucharew H, Campbell J, Millard S, Molina P, Klein J . Computed tomography correlates with pulmonary exacerbations in children with cystic fibrosis. Am J Respir Crit Care Med. 2005; 172(9):1128-32. DOI: 10.1164/rccm.200407-989OC. View

12.

Barker P, Gillie D, Schechter M, Rubin B . Effect of macrolides on in vivo ion transport across cystic fibrosis nasal epithelium. Am J Respir Crit Care Med. 2005; 171(8):868-71. DOI: 10.1164/rccm.200311-1508OC. View

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Pedemonte N, Lukacs G, Du K, Caci E, Zegarra-Moran O, Galietta L . Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening. J Clin Invest. 2005; 115(9):2564-71. PMC: 1190372. DOI: 10.1172/JCI24898. View

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Brennan A, Gyi K, Wood D, Hodson M, Geddes D, Baker E . Relationship between glycosylated haemoglobin and mean plasma glucose concentration in cystic fibrosis. J Cyst Fibros. 2005; 5(1):27-31. DOI: 10.1016/j.jcf.2005.09.001. View

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Saiman L, Mayer-Hamblett N, Campbell P, Marshall B . Heterogeneity of treatment response to azithromycin in patients with cystic fibrosis. Am J Respir Crit Care Med. 2005; 172(8):1008-12. DOI: 10.1164/rccm.200502-218OC. View

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Matsui H, Verghese M, Kesimer M, Schwab U, Randell S, Sheehan J . Reduced three-dimensional motility in dehydrated airway mucus prevents neutrophil capture and killing bacteria on airway epithelial surfaces. J Immunol. 2005; 175(2):1090-9. DOI: 10.4049/jimmunol.175.2.1090. View

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Kulich M, Rosenfeld M, Campbell J, Kronmal R, Gibson R, Goss C . Disease-specific reference equations for lung function in patients with cystic fibrosis. Am J Respir Crit Care Med. 2005; 172(7):885-91. DOI: 10.1164/rccm.200410-1335OC. View

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Martinez T, Llapur C, Williams T, Coates C, Gunderman R, Cohen M . High-resolution computed tomography imaging of airway disease in infants with cystic fibrosis. Am J Respir Crit Care Med. 2005; 172(9):1133-8. PMC: 2718397. DOI: 10.1164/rccm.200412-1665OC. View

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Kraemer R, Blum A, Schibler A, Ammann R, Gallati S . Ventilation inhomogeneities in relation to standard lung function in patients with cystic fibrosis. Am J Respir Crit Care Med. 2004; 171(4):371-8. DOI: 10.1164/rccm.200407-948OC. View

20.

Davies M, Geddes D, Alton E . The effect of varying tonicity on nasal epithelial ion transport in cystic fibrosis. Am J Respir Crit Care Med. 2004; 171(7):760-3. DOI: 10.1164/rccm.200310-1423OC. View