Camostat attenuates airway epithelial sodium channel function in vivo through the inhibition of a channel-activating protease

Journal of Pharmacology and Experimental Therapeutics

Volumn 329, Issue 2, 2009, Pages 764-774

  Coote, K ^a   Atherton Watson, H C ^a   Sugar, R ^a   Young, A ^a   MacKenzie Beevor, A ^a   Gosling, M ^a   Bhalay, G ^a   Bloomfield, G ^a   Dunstan, A ^a   Bridges, R J ^b   Sabater, J R ^c   Abraham, W M ^c   Tully, D ^d   Pacoma, R ^d   Schumacher, A ^d   Harris, J ^d   Danahay, H ^a  

^a NOVARTIS INSTITUTES FOR BIOMEDICAL RESEARCH   (United States)

^b Rosalind Franklin Institute   (United States)

^c MOUNT SINAI MEDICAL CENTER   (United States)

^d Genomic Institute of the Novetis Foundation   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

4 GUANIDINOBENZOIC ACID 4 CARBOXYMETHYL PHENYL ESTER; ALPHA 1 ANTITRYPSIN; APROTININ; CAMOSTAT; CARBOXYLIC ACID DERIVATIVE; EPITHELIAL SODIUM CHANNEL; MATRIPTASE; PROSTASIN; PROTEINASE; SOYBEAN TRYPSIN INHIBITOR; TRYPSIN; ULINASTATIN; UNCLASSIFIED DRUG; CAMOSTAT MESILATE; DRUG DERIVATIVE; GABEXATE; PEPTIDE HYDROLASE; PROTEINASE INHIBITOR;

AEROSOL; ANIMAL EXPERIMENT; ARTICLE; BRONCHUS MUCOSA; CHANNEL GATING; CONTROLLED STUDY; DRUG POTENCY; ENZYME INHIBITION; EPITHELIUM CELL; GUINEA PIG; HUMAN; HUMAN CELL; IC 50; IN VITRO STUDY; IN VIVO STUDY; MALE; MOLECULAR WEIGHT; MUCOCILIARY CLEARANCE; NONHUMAN; PRIORITY JOURNAL; PROTEIN FUNCTION; REGULATORY MECHANISM; RESPIRATORY EPITHELIUM; SHEEP; SINGLE DRUG DOSE; TRACHEA; TRANSLATION REGULATION; ANIMAL; BRONCHUS; CELL CULTURE; CELL MEMBRANE POTENTIAL; CYTOLOGY; DRUG EFFECT; ENZYMOLOGY; METABOLISM; RESPIRATORY TRACT MUCOSA;

ANIMALS; BRONCHI; CELLS, CULTURED; EPITHELIAL CELLS; EPITHELIAL SODIUM CHANNEL; GABEXATE; GUINEA PIGS; HUMANS; MALE; MEMBRANE POTENTIALS; MUCOCILIARY CLEARANCE; PEPTIDE HYDROLASES; PROTEASE INHIBITORS; RESPIRATORY MUCOSA; SHEEP; TRACHEA;

EID: 65649148597     PISSN: 00223565     EISSN: 15210103     Source Type: Journal    
DOI: 10.1124/jpet.108.148155     Document Type: Article

References (38)

1. App EM, King M, Helfesrieder R, Köhler D, and Matthys H (1990) Acute and long-term amiloride inhalation in cystic fibrosis lung disease. A rational approach to cystic fibrosis therapy. Am Rev Respir Dis 141:605-612.
2. Atherton HC, Jones G, and Danahay H (2003) IL-13-induced changes in the goblet cell density of human bronchial epithelial cell cultures: MAP kinase and phospha-tidylinositol 3-kinase regulation. Am J Physiol Lung Cell Mol Physiol 285:L73O-L739.
3. Boucher RC (2007) Airway surface dehydration in cystic fibrosis: pathogenesis and therapy. Annu Rev Med 58:157-170.
4. Boudreault A, Gauthier D, and Lazure C (1998) Proprotein convertase PCl/3-related peptides are potent slow tight-binding inhibitors of murine PCl/3 and Hfurin. J Biol Chem 273:31574-31580.
5. Bowler IM, Kelman B, Worthington D, Littlewood JM, Watson A, Conway SP, Smye SW, James SL, and Sheldon TA (1995) Nebulised amiloride in respiratory exacerbations of cystic fibrosis: a randomised controlled trial. Arch Dis Child 73:427-430.
6. Bridges RJ, Newton BB, Pilewski JM, Devor DC, Poll CT, and Hall RL (2001) Na+ transport in normal and CF human bronchial epithelial cells is inhibited by BAY 39-9437. Am J Physiol Lung Cell Mol Physiol 281:LI6-L23.
7. Burrows E, Southern KW, and Noone P (2006) Sodium channel blockers for cystic fibrosis. Cochrane Database Syst Rev l9;3:CDOO5O87.
8. Caldwell RA, Boucher RC, and Stutts MJ (2005) Neutrophil elastase activates near-silent epithelial Na+ channels and increases airway epithelial Na+ transport. Am J Physiol Lung Cell Mol Physiol 288:L8I3-L8I9.
9. Coote KJ, Atherton H, Young A, Sugar R, Burrows R, Smith NJ, Schlaeppi JM, Groot-Kormelink PJ, Gosling M, and Danahay H (2008) The guinea pig tracheal potential difference as an in vivo model for the study of epithelial sodium channel function in the airways. Br J Pharmacol 155:1025-1033.
10. Davies JC, Davies M, McShane D, Smith S, Chadwick S, Jaffe A, Farley R, Collins L, Bush A, Scallon M, et al. (2005) Potential difference measurements in the lower airway of children with and without cystic fibrosis. Am J Respir Crit Care Med 171:1015-1019.
11. Devor DC, Bridges RJ, and Pilewski JM (2000) Pharmacological modulation of ion transport across wild-type and DeltaF5O8 CFTR-exp res sing human bronchial epithelia. Am J Physiol Cell Physiol 279:C46l-C479.
12. Donaldson SH, Hirsh A, Li DC, Holloway G, Chao J, Boucher RC, and Gabriel SE (2002) Regulation of the epithelial sodium channel by serine proteases in human airways. J Biol Chem 277:8338-8345.
13. Donaldson SH, Bennett WD, Zeman KL, Knowles MR, Tarran R, and Boucher RC (2006) Mucus clearance and lung function in cystic fibrosis with hypertonic saline. N Engl J Med 354:241-250.
14. Elkins MR, Robinson M, Rose BR, Harbour C, Moriarty CP, Marks GB, Belousova EG, Xuan W, and Bye PT; National Hypertonic Saline in Cystic Fibrosis (NHSCF) Study Group (2006) A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis. N Engl J Med 354:229-240.
15. Friedrich R, Fuentes-Prior P, Ong E, Coombs G, Hunter M, Oehler R, Pierson D, Gonzalez R, Huber R, Bode W, et al. (2002) Catalytic domain structures of MT-SPl/matriptase, a matrix-degrading transmembrane serine proteinase. J Biol Chem 277:2160-2168.
16. Graham A, Hasani A, Alton EW, Martin GP, Marriott C, Hodson ME, Clarke SW, and Geddes DM (1993) No added benefit from nebulized amiloride in patients with cystic fibrosis. Eur Respir J 6:1243-1248.
17. Hirsh AJ, Sabater JR, Zamurs A, Smith RT, Paradiso AM, Hopkins S, Abraham WM, and Boucher RC (2004) Evaluation of second generation amiloride analogs as therapy for cystic fibrosis lung disease. J Pharmacol Exp Ther 311:929-938.
18. Hirsh AJ, Zhang J, Zamurs A, Fleegle J, Thelin WR, Caldwell RA, Sabater JR, Abraham WM, Donowitz M, Cha B, et al. (2008) Pharmacological properties of N-(3,5-diamino-6-chloropyrazine-2-carbonyl)-N;'-4-[4-(2,3-dihydroxypropoxy) phe-nyl]butyl-guanidine methanesulfonate (552-02), a novel epithelial sodium channel blocker with potential clinical efficacy for cystic fibrosis lung disease. J Pharmacol Exp Ther 325:77-88.
19. Hughey RP, Bruns JB, Kinlough CL, Harkleroad KL, Tong Q, Carattino MD, Johnson JP, Stockand JD, and Kleyman TR (2004) Epithelial sodium channels are activated by furin-dependent proteolysis. J Biol Chem 279:18111-18114.
20. Knowles M, Gatzy J, and Boucher R (1981) Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis. N Engl J Med 305:1489-1495.
21. Knowles MR, Church NL, Waltner WE, Yankaskas JR, Gilligan P, King M, Edwards LJ, Helms RW, and Boucher RC (1990) A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis. N Engl J Med 322:1189-1194.
22. Köhler D, App E, Schmitz-Schumann M, Würtemberger G, and Matthys H (1986) Inhalation of amiloride improves the mucociliary and the cough clearance in patients with cystic fibroses. Eur J Respir Dis Suppl 146:319-326.
23. List K, Hobson JP, Molinolo A, and Bugge TH (2007) Co-localization of the channel activating protease prostasin/(CAPl/PRSS8) with its candidate activator, matriptase. J Cell Physiol 213:237-245.
24. Livraghi A and Randell SH (2007) Cystic fibrosis and other respiratory diseases of impaired mucus clearance. Toxicol Pathol 35:116-129.
25. Mentz WM, Brown JB, Friedman M, Stutts MJ, Gatzy JT, and Boucher RC (1986) Deposition, clearance, and effects of aerosolized amiloride in sheep airways. Am Rev Respir Dis 134:938-943.
26. Muto S, Imai M, and Asano Y (1994) Mechanisms of the hyperkalemia caused by nafamostat mesilate: effects of its two metabolites on Na+ and K+ transport properties in the rabbit cortical collecting duct. Br J Pharmacol 111:173-178.
27. Myerburg MM, McKenna EE, Luke CJ, Frizzell RA, Kleyman TR, and Pilewski JM (2008) Prostasin expression is regulated by airway surface liquid volume and is increased in cystic fibrosis. Am J Physiol Lung Cell Mol Physiol 294:L932-L94l.
28. Noone PG, Regnis JA, Liu X, Brouwer KL, Robinson M, Edwards L, and Knowles MR (1997) Airway deposition and clearance and systemic pharmacokinetics of amiloride following aerosolization with an ultrasonic nebulizer to normal airways. Chest 112:1283-1290.
29. Ordonez CL, Henig NR, Mayer-Hamblett N, Accurso FJ, Burns JL, Chmiel JF, Daines CL, Gibson RL, McNamara S, Retsch-Bogart GZ, et al. (2003) Inflammatory and microbiologic markers in induced sputum after intravenous antibiotics in cystic fibrosis. Am J Respir Crit Care Med 168:1471-1475.
30. Picard N, Eladari D, El Moghrabi S, Planes C, Bourgeois S, Houillier P, Wang Q, Burnier M, Deschenes G, Knepper MA, et al. (2008) Defective ENaC processing and function in tissue kallikrein-deficient mice. J Biol Chem 283:4602-4611.
31. Planes C and Caughey GH (2007) Regulation of the epithelial Na+ channel by peptidases. Curr Top Dev Biol 78:23-46.
32. Quinton PM (1989) Defective epithelial ion transport in cystic fibrosis. Clin Chem 35:726-730.
33. Ramjee MK, Henderson IM, McLoughlin SB, and Padova A (2000) The kinetic and structural characterization of the reaction of nafamostat with bovine pancreatic trypsin. Thromb Res 98:559-569.
34. Ratjen F and Dòring G (2003) Cystic fibrosis. Lancet 361:681-689.
35. Shaul PW, North AJ, Wu LC, Wells LB, Brannon TS, Lau KS, Michel T, Margraf LR, and Star RA (1994) Endothelial nitric oxide synthase is expressed in cultured human bronchiolar epithelium. J Clin Invest 94:2231-2236.
36. Shipway A, Danahay H, Williams JA, Tully DC, Backes BJ, and Harris JL (2004) Biochemical characterization of prostasin, a channel activating protease. Biochem Biophys Res Commun 324:953-963.
37. Tong Z, Illek B, Bhagwandin VJ, Verghese GM, and Caughey GH (2004) Prostasin, a membrane-anchored serine peptidase, regulates sodium currents in JME/CF15 cells, a cystic fibrosis airway epithelial cell line. Am J Physiol Lung Cell Mol Physiol 287:L928-L935.
38. Vallet V, Chraibi A, Gaeggeler HP, Horisberger JD, and Rossier BC (1997) An epithelial serine protease activates the amiloride-sensitive sodium channel. Nature 389:607-610.