Targeting Proteases in Cystic Fibrosis Lung Disease. Paradigms, Progress, and Potential

Overview

Journal Am J Respir Crit Care Med

Specialty Critical Care

Date 2019 Oct 19

PMID 31626562

Citations 33

Authors

Michael C McKelvey

Sinead Weldon

Daniel F McAuley

Marcus A Mall

Clifford C Taggart

Affiliations

Soon will be listed here.

Citing Articles

Exploring Proteases as Alternative Molecular Targets to Tackle Inflammation in Cystic Fibrosis Respiratory Infections.

Sandri A, Boschi F Int J Mol Sci. 2025; 26(5).

PMID: 40076497 PMC: 11899166. DOI: 10.3390/ijms26051871.

Targeting neutrophil serine proteases in bronchiectasis.

Chalmers J, Mall M, Chotirmall S, ODonnell A, Flume P, Hasegawa N Eur Respir J. 2024; 65(1).

PMID: 39467608 PMC: 11694565. DOI: 10.1183/13993003.01050-2024.

Gene expression responses of CF airway epithelial cells exposed to elexacaftor/tezacaftor/ivacaftor suggest benefits beyond improved CFTR channel function.

Hampton T, Barnaby R, Roche C, Nymon A, Fukutani K, MacKenzie T Am J Physiol Lung Cell Mol Physiol. 2024; 327(6):L905-L916.

PMID: 39437760 PMC: 11684945. DOI: 10.1152/ajplung.00272.2024.

Cystic fibrosis.

Mall M, Burgel P, Castellani C, Davies J, Salathe M, Taylor-Cousar J Nat Rev Dis Primers. 2024; 10(1):53.

PMID: 39117676 DOI: 10.1038/s41572-024-00538-6.

Neutrophil Elastase Degrades Histone Deacetylases and Sirtuin 1 in Primary Human Monocyte Derived Macrophages.

Zheng S, Bulut G, Kummarapurugu A, Ma J, Voynow J Int J Mol Sci. 2024; 25(8).

PMID: 38673851 PMC: 11050352. DOI: 10.3390/ijms25084265.

References

Greenlee K, Werb Z, Kheradmand F . Matrix metalloproteinases in lung: multiple, multifarious, and multifaceted. Physiol Rev. 2007; 87(1):69-98. PMC: 2656382. DOI: 10.1152/physrev.00022.2006. View

Hisert K, Heltshe S, Pope C, Jorth P, Wu X, Edwards R . Restoring Cystic Fibrosis Transmembrane Conductance Regulator Function Reduces Airway Bacteria and Inflammation in People with Cystic Fibrosis and Chronic Lung Infections. Am J Respir Crit Care Med. 2017; 195(12):1617-1628. PMC: 5476912. DOI: 10.1164/rccm.201609-1954OC. View

Dittrich A, Kuhbandner I, Gehrig S, Rickert-Zacharias V, Twigg M, Wege S . Elastase activity on sputum neutrophils correlates with severity of lung disease in cystic fibrosis. Eur Respir J. 2018; 51(3). DOI: 10.1183/13993003.01910-2017. View

Rubio F, Cooley J, Accurso F, Remold-ODonnell E . Linkage of neutrophil serine proteases and decreased surfactant protein-A (SP-A) levels in inflammatory lung disease. Thorax. 2004; 59(4):318-23. PMC: 1763800. DOI: 10.1136/thx.2003.014902. View

Haerteis S, Krappitz M, Bertog M, Krappitz A, Baraznenok V, Henderson I . Proteolytic activation of the epithelial sodium channel (ENaC) by the cysteine protease cathepsin-S. Pflugers Arch. 2012; 464(4):353-65. PMC: 3448907. DOI: 10.1007/s00424-012-1138-3. View

Rao N, Argyle B, Xu X, Reynolds P, Walenga J, Prechel M . Low anticoagulant heparin targets multiple sites of inflammation, suppresses heparin-induced thrombocytopenia, and inhibits interaction of RAGE with its ligands. Am J Physiol Cell Physiol. 2010; 299(1):C97-110. DOI: 10.1152/ajpcell.00009.2010. View

Turnbull A, Pyle C, Patel D, Jackson P, Hilliard T, Regamey N . Abnormal pro-gly-pro pathway and airway neutrophilia in pediatric cystic fibrosis. J Cyst Fibros. 2019; 19(1):40-48. PMC: 7001103. DOI: 10.1016/j.jcf.2019.05.017. View

Hahn I, Klaus A, Janze A, Steinwede K, Ding N, Bohling J . Cathepsin G and neutrophil elastase play critical and nonredundant roles in lung-protective immunity against Streptococcus pneumoniae in mice. Infect Immun. 2011; 79(12):4893-901. PMC: 3232647. DOI: 10.1128/IAI.05593-11. View

Keating D, Marigowda G, Burr L, Daines C, Mall M, McKone E . VX-445-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and One or Two Phe508del Alleles. N Engl J Med. 2018; 379(17):1612-1620. PMC: 6289290. DOI: 10.1056/NEJMoa1807120. View

10.

Xu X, Abdalla T, Bratcher P, Jackson P, Sabbatini G, Wells J . Doxycycline improves clinical outcomes during cystic fibrosis exacerbations. Eur Respir J. 2017; 49(4). DOI: 10.1183/13993003.01102-2016. View

11.

Padrines M, Wolf M, Walz A, Baggiolini M . Interleukin-8 processing by neutrophil elastase, cathepsin G and proteinase-3. FEBS Lett. 1994; 352(2):231-5. DOI: 10.1016/0014-5793(94)00952-x. View

12.

Akthar S, Patel D, Beale R, Peiro T, Xu X, Gaggar A . Matrikines are key regulators in modulating the amplitude of lung inflammation in acute pulmonary infection. Nat Commun. 2015; 6:8423. PMC: 4595997. DOI: 10.1038/ncomms9423. View

13.

Voynow J, Fischer B, Zheng S . Proteases and cystic fibrosis. Int J Biochem Cell Biol. 2008; 40(6-7):1238-45. PMC: 2431113. DOI: 10.1016/j.biocel.2008.03.003. View

14.

Dubois A, Gauthier A, Brea D, Varaigne F, Diot P, Gauthier F . Influence of DNA on the activities and inhibition of neutrophil serine proteases in cystic fibrosis sputum. Am J Respir Cell Mol Biol. 2012; 47(1):80-6. DOI: 10.1165/rcmb.2011-0380OC. View

15.

Griffin K, Fischer B, Kummarapurugu A, Zheng S, Kennedy T, Rao N . 2-O, 3-O-desulfated heparin inhibits neutrophil elastase-induced HMGB-1 secretion and airway inflammation. Am J Respir Cell Mol Biol. 2013; 50(4):684-9. PMC: 4068925. DOI: 10.1165/rcmb.2013-0338RC. View

16.

Weldon S, McNally P, McElvaney N, Elborn J, McAuley D, Wartelle J . Decreased levels of secretory leucoprotease inhibitor in the Pseudomonas-infected cystic fibrosis lung are due to neutrophil elastase degradation. J Immunol. 2009; 183(12):8148-56. PMC: 3404409. DOI: 10.4049/jimmunol.0901716. View

17.

Rogan M, Taggart C, M Greene C, Murphy P, ONeill S, McElvaney N . Loss of microbicidal activity and increased formation of biofilm due to decreased lactoferrin activity in patients with cystic fibrosis. J Infect Dis. 2004; 190(7):1245-53. DOI: 10.1086/423821. View

18.

Wielputz M, Eichinger M, Biederer J, Wege S, Stahl M, Sommerburg O . Imaging of Cystic Fibrosis Lung Disease and Clinical Interpretation. Rofo. 2016; 188(9):834-45. DOI: 10.1055/s-0042-104936. View

19.

Gehrig S, Mall M, Schultz C . Spatially resolved monitoring of neutrophil elastase activity with ratiometric fluorescent reporters. Angew Chem Int Ed Engl. 2012; 51(25):6258-61. DOI: 10.1002/anie.201109226. View

20.

Hirche T, Benabid R, Deslee G, Gangloff S, Achilefu S, Guenounou M . Neutrophil elastase mediates innate host protection against Pseudomonas aeruginosa. J Immunol. 2008; 181(7):4945-54. DOI: 10.4049/jimmunol.181.7.4945. View