Novel therapeutic approaches for pulmonary fibrosis

British Journal of Pharmacology

Volumn 163, Issue 1, 2011, Pages 141-172

  Datta, Arnab ^a   Scotton, Chris J ^a   Chambers, Rachel C ^a  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

drug targets; fibrosis therapy; myofibroblast; pulmonary fibrosis

Indexed keywords

ACETYLCYSTEINE; AMBRISENTAN; ANTIFIBROTIC AGENT; AZATHIOPRINE; BOSENTAN; CORTICOSTEROID; CYCLOPHOSPHAMIDE; ETANERCEPT; FG 3019; FRESOLIMUMAB; GAMMA INTERFERON 1B; HEPARIN; ILOPROST; IMATINIB; INTERLEUKIN 13 ANTIBODY; LOSARTAN; MINOCYCLINE; MONOCYTE CHEMOTACTIC PROTEIN 1 INHIBITOR; PIRFENIDONE; PLACEBO; PREDNISOLONE; QAX 576; SILDENAFIL; SOMATOSTATIN DERIVATIVE; STX 100; TETRATHIOMOLYBDIC ACID; THALIDOMIDE; TRANSFORMING GROWTH FACTOR BETA ANTIBODY; UNCLASSIFIED DRUG; UNINDEXED DRUG; WARFARIN; ZILEUTON;

CELL CYCLE; CELL DAMAGE; CELL INTERACTION; DNA POLYMORPHISM; DRUG DOSE COMPARISON; DRUG MECHANISM; DRUG MEGADOSE; FIBROGENESIS; FIBROSING ALVEOLITIS; HUMAN; LOW DRUG DOSE; LUNG ALVEOLUS EPITHELIUM; LUNG FIBROSIS; LUNG SARCOIDOSIS; MESENCHYME CELL; MOLECULARLY TARGETED THERAPY; NONHUMAN; PATHOGENESIS; PHASE 1 CLINICAL TRIAL (TOPIC); PNEUMONIA; PRIORITY JOURNAL; PROGNOSIS; RANDOMIZED CONTROLLED TRIAL (TOPIC); RESPIRATORY FAILURE; REVIEW; RISK FACTOR; SURVIVAL TIME; SYSTEMIC SCLEROSIS; VIRUS INFECTION; WOUND HEALING;

ANIMALS; CLINICAL TRIALS AS TOPIC; HUMANS; IDIOPATHIC PULMONARY FIBROSIS; MOLECULAR TARGETED THERAPY;

EID: 79953728756     PISSN: 00071188     EISSN: 14765381     Source Type: Journal    
DOI: 10.1111/j.1476-5381.2011.01247.x     Document Type: Review

References (286)

1. Adler KB, Low RB, Leslie KO, Mitchell J, Evans JN, (1989). Contractile cells in normal and fibrotic lung. Lab Invest 60: 473-485. (Pubitemid 19141795)
2. Aguilar S, Scotton CJ, McNulty K, Nye E, Stamp G, Laurent G, et al,. (2009). Bone marrow stem cells expressing keratinocyte growth factor via an inducible lentivirus protects against bleomycin-induced pulmonary fibrosis. PloS ONE 4: e8013.
3. Akiri G, Sabo E, Dafni H, Vadasz Z, Kartvelishvily Y, Gan N, et al,. (2003). Lysyl oxidase-related protein-1 promotes tumor fibrosis and tumor progression in vivo. Cancer Res 63: 1657-1666. (Pubitemid 36373659)
4. Allen JT, Knight RA, Bloor CA, Spiteri MA, (1999). Enhanced insulin-like growth factor binding protein-related protein 2 (Connective tissue growth factor) expression in patients with idiopathic pulmonary fibrosis and pulmonary sarcoidosis. Am J Respir Cell Mol Biol 21: 693-700. (Pubitemid 129502288)
5. Andersson-Sjoland A, de Alba CG, Nihlberg K, Becerril C, Ramirez R, Pardo A, et al,. (2008). Fibrocytes are a potential source of lung fibroblasts in idiopathic pulmonary fibrosis. Int J Biochem Cell Biol 40: 2129-2140.
6. Antoniades HN, Bravo MA, Avila RE, Galanopoulos T, Neville-Golden J, Maxwell M, et al,. (1990). Platelet-derived growth factor in idiopathic pulmonary fibrosis. J Clin Invest 86: 1055-1064. (Pubitemid 20370408)
7. Antoniu SA, (2008). Somatostatin analogs for idiopathic pulmonary fibrosis therapy. Expert Opin Investig Drugs 17: 1137-1140.
8. Aono Y, Nishioka Y, Inayama M, Ugai M, Kishi J, Uehara H, et al,. (2005). Imatinib as a novel antifibrotic agent in bleomycin-induced pulmonary fibrosis in mice. Am J Respir Crit Care Med 171: 1279-1285. (Pubitemid 40740736)
9. Aoyagi-Ikeda K, Maeno T, Matsui H, Ueno M, Hara K, Aoki Y, et al,. (2010). Notch Induces myofibroblast differentiation of alveolar epithelial cells via TGF-ss/Smad3 pathway. Am J Respir Cell Mol Biol (in press).
10. Ask K, Bonniaud P, Maass K, Eickelberg O, Margetts PJ, Warburton D, et al,. (2008). Progressive pulmonary fibrosis is mediated by TGF-beta isoform 1 but not TGF-beta3. Int J Biochem Cell Biol 40: 484-495. (Pubitemid 351249886)
11. Atabai K, Jame S, Azhar N, Kuo A, Lam M, McKleroy W, et al,. (2009). Mfge8 diminishes the severity of tissue fibrosis in mice by binding and targeting collagen for uptake by macrophages. J Clin Invest 119: 3713-3722.
12. ATS/ERS (2000). American Thoracic Society. Idiopathic pulmonary fibrosis: diagnosis and treatment. International consensus statement. American Thoracic Society (ATS), and the European Respiratory Society (ERS). Am J Respir Crit Care Med 161: 646-664.
13. Avivi-Green C, Singal M, Vogel WF, (2006). Discoidin domain receptor 1-deficient mice are resistant to bleomycin-induced lung fibrosis. Am J Respir Crit Care Med 174: 420-427. (Pubitemid 44223123)
14. Baran CP, Opalek JM, McMaken S, Newland CA, O'Brien JM Jr, Hunter MG, et al,. (2007). Important roles for macrophage colony-stimulating factor, CC chemokine ligand 2, and mononuclear phagocytes in the pathogenesis of pulmonary fibrosis. Am J Respir Crit Care Med 176: 78-89. (Pubitemid 47048057)
15. Barbas-Filho JV, Ferreira MA, Sesso A, Kairalla RA, Carvalho CR, Capelozzi VL, (2001). Evidence of type II pneumocyte apoptosis in the pathogenesis of idiopathic pulmonary fibrosis (IFP)/usual interstitial pneumonia (UIP). J Clin Pathol 54: 132-138. (Pubitemid 32128415)
16. Barry-Hamilton V, Spangler R, Marshall D, McCauley S, Rodriguez HM, Oyasu M, et al,. (2010). Allosteric inhibition of lysyl oxidase-like-2 impedes the development of a pathologic microenvironment. Nat Med 16: 1009-1017.
17. Bauman KA, Wettlaufer SH, Okunishi K, Vannella KM, Stoolman JS, Huang SK, et al,. (2010). The antifibrotic effects of plasminogen activation occur via prostaglandin E2 synthesis in humans and mice. J Clin Invest 120: 1950-1960.
18. Beeh KM, Beier J, Haas IC, Kornmann O, Micke P, Buhl R, (2002). Glutathione deficiency of the lower respiratory tract in patients with idiopathic pulmonary fibrosis. Eur Respir J 19: 1119-1123. (Pubitemid 34649628)
19. Behr J, Noble PW, (2009). Clinical trials in interstitial lung disease. European Respiratory Soc Monograph 46: 67-84.
20. Behr J, Degenkolb B, Maier K, Braun B, Beinert T, Krombach F, et al,. (1995). Increased oxidation of extracellular glutathione by bronchoalveolar inflammatory cells in diffuse fibrosing alveolitis. Eur Respir J 8: 1286-1292.
21. Behr J, Degenkolb B, Krombach F, Vogelmeier C, (2002). Intracellular glutathione and bronchoalveolar cells in fibrosing alveolitis: effects of N-acetylcysteine. Eur Respir J 19: 906-911. (Pubitemid 34567906)
22. Belperio JA, Dy M, Burdick MD, Xue YY, Li K, Elias JA, et al,. (2002). Interaction of IL-13 and C10 in the pathogenesis of bleomycin-induced pulmonary fibrosis. Am J Respir Cell Mol Biol 27: 419-427.
23. Bitterman PB, Wewers MD, Rennard SI, Adelberg S, Crystal RG, (1986). Modulation of alveolar macrophage-driven fibroblast proliferation by alternative macrophage mediators. J Clin Invest 77: 700-708. (Pubitemid 16067939)
24. Blanc-Brude OP, Archer F, Leoni P, Derian C, Bolsover S, Laurent GJ, et al,. (2005). Factor Xa stimulates fibroblast procollagen production, proliferation, and calcium signaling via PAR1 activation. Exp Cell Res 304: 16-27. (Pubitemid 40222073)
25. Bonniaud P, Margetts PJ, Kolb M, Haberberger T, Kelly M, Robertson J, et al,. (2003). Adenoviral gene transfer of connective tissue growth factor in the lung induces transient fibrosis. Am J Respir Crit Care Med 168: 770-778. (Pubitemid 37188299)
26. Bonniaud P, Martin G, Margetts PJ, Ask K, Robertson J, Gauldie J, et al,. (2004). Connective tissue growth factor is crucial to inducing a profibrotic environment in 'fibrosis-resistant' BALB/c mouse lungs. Am J Respir Cell Mol Biol 31: 510-516. (Pubitemid 39463399)
27. Bonniaud P, Margetts PJ, Kolb M, Schroeder JA, Kapoun AM, Damm D, et al,. (2005). Progressive transforming growth factor beta1-induced lung fibrosis is blocked by an orally active ALK5 kinase inhibitor. Am J Respir Crit Care Med 171: 889-898. (Pubitemid 40562720)
28. Borensztajn K, Bresser P, van der Loos C, Bot I, van den Blink B, den Bakker MA, et al,. (2010). Protease-activated receptor-2 induces myofibroblast differentiation and tissue factor up-regulation during bleomycin-induced lung injury. Potential role in pulmonary fibrosis. Am J Pathol 177: 2753-2764.
29. Borie R, Fabre A, Prost F, Marchal-Somme J, Lebtahi R, Marchand-Adam S, et al,. (2008). Activation of somatostatin receptors attenuates pulmonary fibrosis. Thorax 63: 251-258. (Pubitemid 351373721)
30. Borok Z, Buhl R, Grimes GJ, Bokser AD, Hubbard RC, Holroyd KJ, et al,. (1991a). Effect of glutathione aerosol on oxidant-antioxidant imbalance in idiopathic pulmonary fibrosis. Lancet 338: 215-216.
31. Borok Z, Gillissen A, Buhl R, Hoyt RF, Hubbard RC, Ozaki T, et al,. (1991b). Augmentation of functional prostaglandin E levels on the respiratory epithelial surface by aerosol administration of prostaglandin E. Am Rev Respir Dis 144: 1080-1084.
32. Borowski A, Kuepper M, Horn U, Knupfer U, Zissel G, Hohne K, et al,. (2008). Interleukin-13 acts as an apoptotic effector on lung epithelial cells and induces pro-fibrotic gene expression in lung fibroblasts. Clin Exp Allergy 38: 619-628. (Pubitemid 351406073)
33. Breuss JM, Gallo J, DeLisser HM, Klimanskaya IV, Folkesson HG, Pittet JF, et al,. (1995). Expression of the beta 6 integrin subunit in development, neoplasia and tissue repair suggests a role in epithelial remodeling. J Cell Sci 108: 2241-2251.
34. Brewer GJ, Ullenbruch MR, Dick R, Olivarez L, Phan SH, (2003). Tetrathiomolybdate therapy protects against bleomycin-induced pulmonary fibrosis in mice. J Lab Clin Med 141: 210-216. (Pubitemid 36324239)
35. Brewer GJ, Dick R, Ullenbruch MR, Jin H, Phan SH, (2004). Inhibition of key cytokines by tetrathiomolybdate in the bleomycin model of pulmonary fibrosis. J Inorg Biochem 98: 2160-2167. (Pubitemid 39488145)
36. Bucala R, Spiegel LA, Chesney J, Hogan M, Cerami A, (1994). Circulating fibrocytes define a new leukocyte subpopulation that mediates tissue repair. Mol Med 1: 71-81.
37. Burdick MD, Murray LA, Keane MP, Xue YY, Zisman DA, Belperio JA, et al,. (2005). CXCL11 attenuates bleomycin-induced pulmonary fibrosis via inhibition of vascular remodeling. Am J Respir Crit Care Med 171: 261-268.
38. Cantin AM, Hubbard RC, Crystal RG, (1989). Glutathione deficiency in the epithelial lining fluid of the lower respiratory tract in idiopathic pulmonary fibrosis. Am Rev Respir Dis 139: 370-372. (Pubitemid 19057410)
39. Chambers RC, (2008). Procoagulant signalling mechanisms in lung inflammation and fibrosis: novel opportunities for pharmacological intervention? Br J Pharmacol 153 (Suppl. 1): S367-S378. (Pubitemid 351340998)
40. Chambers RC, Leoni P, Blanc-Brude OP, Wembridge DE, Laurent GJ, (2000). Thrombin is a potent inducer of connective tissue growth factor production via proteolytic activation of protease-activated receptor-1. J Biol Chem 275: 35584-35591.
41. Chambers RC, Leoni P, Kaminski N, Laurent GJ, Heller RA, (2003). Global expression profiling of fibroblast responses to transforming growth factor-beta1 reveals the induction of inhibitor of differentiation-1 and provides evidence of smooth muscle cell phenotypic switching. Am J Pathol 162: 533-546. (Pubitemid 36267860)
42. Chilosi M, Poletti V, Murer B, Lestani M, Cancellieri A, Montagna L, et al,. (2002). Abnormal re-epithelialization and lung remodeling in idiopathic pulmonary fibrosis: the role of deltaN-p63. Lab Invest 82: 1335-1345. (Pubitemid 35191537)
43. Chilosi M, Poletti V, Zamo A, Lestani M, Montagna L, Piccoli P, et al,. (2003). Aberrant Wnt/beta-catenin pathway activation in idiopathic pulmonary fibrosis. Am J Pathol 162: 1495-1502. (Pubitemid 36512831)
44. Cho HY, Reddy SP, Yamamoto M, Kleeberger SR, (2004). The transcription factor NRF2 protects against pulmonary fibrosis. FASEB J 18: 1258-1260. (Pubitemid 39561569)
45. Chrzanowska-Wodnicka M, Burridge K, (1996). Rho-stimulated contractility drives the formation of stress fibers and focal adhesions. J Cell Biol 133: 1403-1415. (Pubitemid 26192339)
46. Clark JG, Madtes DK, Raghu G, (1993). Effects of platelet-derived growth factor isoforms on human lung fibroblast proliferation and procollagen gene expression. Exp Lung Res 19: 327-344. (Pubitemid 23159765)
47. Coker RK, Laurent GJ, Shahzeidi S, Lympany PA, du Bois RM, Jeffery PK, et al,. (1997). Transforming growth factors-beta 1, -beta 2, and -beta 3 stimulate fibroblast procollagen production in vitro but are differentially expressed during bleomycin-induced lung fibrosis. Am J Pathol 150: 981-991.
48. Collard HR, (2010). Idiopathic pulmonary fibrosis and pirfenidone. Eur Respir J 35: 728-729.
49. Collard HR, Ryu JH, Douglas WW, Schwarz MI, Curran-Everett D, King TE, et al,. (2004). Combined corticosteroid and cyclophosphamide therapy does not alter survival in idiopathic pulmonary fibrosis. Chest 125: 2169-2174. (Pubitemid 38788186)
50. Corrin B, Dewar A, Rodriguez-Roisin R, Turner-Warwick M, (1985). Fine structural changes in cryptogenic fibrosing alveolitis and asbestosis. J Pathol 147: 107-119. (Pubitemid 16220638)
51. Coultas DB, Zumwalt RE, Black WC, Sobonya RE, (1994). The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med 150: 967-972. (Pubitemid 24318021)
52. D'Amato RJ, Loughnan MS, Flynn E, Folkman J, (1994). Thalidomide is an inhibitor of angiogenesis. Proc Natl Acad Sci USA 91: 4082-4085. (Pubitemid 24139577)
53. Daniels CE, Wilkes MC, Edens M, Kottom TJ, Murphy SJ, Limper AH, et al,. (2004). Imatinib mesylate inhibits the profibrogenic activity of TGF-beta and prevents bleomycin-mediated lung fibrosis. J Clin Invest 114: 1308-1316. (Pubitemid 40385302)
54. Daniels CE, Lasky JA, Limper AH, Mieras K, Gabor E, Schroeder DR, (2010). Imatinib treatment for idiopathic pulmonary fibrosis: randomized placebo-controlled trial results. Am J Respir Crit Care Med 181: 604-610.
55. Davies HR, Richeldi L, Walters EH, (2003). Immunomodulatory agents for idiopathic pulmonary fibrosis. Cochrane Database Syst Rev (3): CD003134.
56. Day E, Waters B, Spiegel K, Alnadaf T, Manley PW, Buchdunger E, et al,. (2008). Inhibition of collagen-induced discoidin domain receptor 1 and 2 activation by imatinib, nilotinib and dasatinib. Eur J Pharmacol 599: 44-53.
57. Demedts M, Behr J, Buhl R, Costabel U, Dekhuijzen R, Jansen HM, et al,. (2005). High-dose acetylcysteine in idiopathic pulmonary fibrosis. N Engl J Med 353: 2229-2242. (Pubitemid 41697286)
58. Deterding RR, Jacoby CR, Shannon JM, (1996). Acidic fibroblast growth factor and keratinocyte growth factor stimulate fetal rat pulmonary epithelial growth. Am J Physiol 271: L495-L505.
59. Deterding RR, Havill AM, Yano T, Middleton SC, Jacoby CR, Shannon JM, et al,. (1997). Prevention of bleomycin-induced lung injury in rats by keratinocyte growth factor. Proc Assoc Am Physicians 109: 254-268. (Pubitemid 27209032)
60. Di Sario A, Bendia E, Svegliati Baroni G, Ridolfi F, Casini A, Ceni E, et al,. (2002). Effect of pirfenidone on rat hepatic stellate cell proliferation and collagen production. J Hepatol 37: 584-591. (Pubitemid 35305537)
61. Ding LW, Wu Z, Liu T, Ullenbruch M, Liu J, Phan S, (2010). Activation of stem cell factor/c-kit signaling pathway in pulmonary fibrosis. Am J Respir Crit Care Med 181 (Suppl.): A3536.
62. Dohi M, Hasegawa T, Yamamoto K, Marshall BC, (2000). Hepatocyte growth factor attenuates collagen accumulation in a murine model of pulmonary fibrosis. Am J Respir Crit Care Med 162: 2302-2307. (Pubitemid 32051704)
63. Dorscheid DR, Wojcik KR, Sun S, Marroquin B, White SR, (2001). Apoptosis of airway epithelial cells induced by corticosteroids. Am J Respir Crit Care Med 164: 1939-1947. (Pubitemid 33152334)
64. Douglas WW, Ryu JH, Bjoraker JA, Schroeder DR, Myers JL, Tazelaar HD, et al,. (1997). Colchicine versus prednisone as treatment of usual interstitial pneumonia. Mayo Clin Proc 72: 201-209. (Pubitemid 28196620)
65. Douglas WW, Ryu JH, Schroeder DR, (2000). Idiopathic pulmonary fibrosis: impact of oxygen and colchicine, prednisone, or no therapy on survival. Am J Respir Crit Care Med 161: 1172-1178. (Pubitemid 30243826)
66. Elias JA, Jimenez SA, Freundlich B, (1987). Recombinant gamma, alpha, and beta interferon regulation of human lung fibroblast proliferation. Am Rev Respir Dis 135: 62-65. (Pubitemid 17223890)
67. Elias JA, Freundlich B, Adams S, Rosenbloom J, (1990). Regulation of human lung fibroblast collagen production by recombinant interleukin-1, tumor necrosis factor, and interferon-gamma. Ann N Y Acad Sci 580: 233-244. (Pubitemid 20170401)
68. Erler JT, Bennewith KL, Nicolau M, Dornhofer N, Kong C, Le QT, et al,. (2006). Lysyl oxidase is essential for hypoxia-induced metastasis. Nature 440: 1222-1226.
69. Ezquerro IJ, Lasarte JJ, Dotor J, Castilla-Cortazar I, Bustos M, Penuelas I, et al,. (2003). A synthetic peptide from transforming growth factor beta type III receptor inhibits liver fibrogenesis in rats with carbon tetrachloride liver injury. Cytokine 22: 12-20. (Pubitemid 37222298)
70. Feghali-Bostwick CA, Tsai CG, Valentine VG, Kantrow S, Stoner MW, Pilewski JM, et al,. (2007). Cellular and humoral autoreactivity in idiopathic pulmonary fibrosis. J Immunol 179: 2592-2599.
71. Fichtner-Feigl S, Strober W, Kawakami K, Puri RK, Kitani A, (2006). IL-13 signaling through the IL-13alpha2 receptor is involved in induction of TGF-beta1 production and fibrosis. Nat Med 12: 99-106. (Pubitemid 43050083)
72. Finch PW, Rubin JS, Miki T, Ron D, Aaronson SA, (1989). Human KGF is FGF-related with properties of a paracrine effector of epithelial cell growth. Science 245: 752-755. (Pubitemid 19214010)
73. Flaherty KR, Toews GB, Lynch JP 3rd, Kazerooni EA, Gross BH, Strawderman RL, et al,. (2001). Steroids in idiopathic pulmonary fibrosis: a prospective assessment of adverse reactions, response to therapy, and survival. Am J Med 110: 278-282. (Pubitemid 32187288)
74. Flaherty KR, Mumford JA, Murray S, Kazerooni EA, Gross BH, Colby TV, et al,. (2003). Prognostic implications of physiologic and radiographic changes in idiopathic interstitial pneumonia. Am J Respir Crit Care Med 168: 543-548. (Pubitemid 37088596)
75. Gasse P, Riteau N, Charron S, Girre S, Fick L, Petrilli V, et al,. (2009). Uric acid is a danger signal activating NALP3 inflammasome in lung injury inflammation and fibrosis. Am J Respir Crit Care Med 179: 903-913.
76. Gharaee-Kermani M, Denholm EM, Phan SH, (1996). Costimulation of fibroblast collagen and transforming growth factor beta1 gene expression by monocyte chemoattractant protein-1 via specific receptors. J Biol Chem 271: 17779-17784. (Pubitemid 26250752)
77. Giaid A, Michel RP, Stewart DJ, Sheppard M, Corrin B, Hamid Q, (1993). Expression of endothelin-1 in lungs of patients with cryptogenic fibrosing alveolitis. Lancet 341: 1550-1554. (Pubitemid 23170459)
78. Giri SN, Leonard S, Shi X, Margolin SB, Vallyathan V, (1999). Effects of pirfenidone on the generation of reactive oxygen species in vitro. J Environ Pathol Toxicol Oncol 18: 169-177.
79. Giri SN, Biring I, Nguyen T, Wang Q, Hyde DM, (2002). Abrogation of bleomycin-induced lung fibrosis by nitric oxide synthase inhibitor, aminoguanidine in mice. Nitric Oxide 7: 109-118. (Pubitemid 35177002)
80. Goldstein RH, Polgar P, (1982). The effect and interaction of bradykinin and prostaglandins on protein and collagen production by lung fibroblasts. J Biol Chem 257: 8630-8633. (Pubitemid 12004184)
81. Gressner AM, Weiskirchen R, (2006). Modern pathogenetic concepts of liver fibrosis suggest stellate cells and TGF-beta as major players and therapeutic targets. J Cell Mol Med 10: 76-99. (Pubitemid 44874293)
82. Gribbin J, Hubbard RB, Le Jeune I, Smith CJ, West J, Tata LJ, (2006). Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UK. Thorax 61: 980-985. (Pubitemid 44787169)
83. Grunert S, Jechlinger M, Beug H, (2003). Diverse cellular and molecular mechanisms contribute to epithelial plasticity and metastasis. Nat Rev Mol Cell Biol 4: 657-665. (Pubitemid 36934968)
84. Gu L, Tseng S, Horner RM, Tam C, Loda M, Rollins BJ, (2000). Control of TH2 polarization by the chemokine monocyte chemoattractant protein-1. Nature 404: 407-411. (Pubitemid 30164344)
85. Gunther A, Lubke N, Ermert M, Schermuly RT, Weissmann N, Breithecker A, et al,. (2003). Prevention of bleomycin-induced lung fibrosis by aerosolization of heparin or urokinase in rabbits. Am J Respir Crit Care Med 168: 1358-1365. (Pubitemid 37487332)
86. Gurujeyalakshmi G, Giri SN, (1995). Molecular mechanisms of antifibrotic effect of interferon gamma in bleomycin-mouse model of lung fibrosis: downregulation of TGF-beta and procollagen I and III gene expression. Exp Lung Res 21: 791-808.
87. Gurujeyalakshmi G, Hollinger MA, Giri SN, (1999). Pirfenidone inhibits PDGF isoforms in bleomycin hamster model of lung fibrosis at the translational level. Am J Physiol 276: L311-L318. (Pubitemid 29102273)
88. Hale ML, Margolin SB, Krakauer T, Roy CJ, Stiles BG, (2002). Pirfenidone blocks the in vitro and in vivo effects of staphylococcal enterotoxin B. Infect Immun 70: 2989-2994. (Pubitemid 34564158)
89. Hamada N, Kuwano K, Yamada M, Hagimoto N, Hiasa K, Egashira K, et al,. (2005). Anti-vascular endothelial growth factor gene therapy attenuates lung injury and fibrosis in mice. J Immunol 175: 1224-1231. (Pubitemid 41095011)
90. Hancock A, Armstrong L, Gama R, Millar A, (1998). Production of interleukin 13 by alveolar macrophages from normal and fibrotic lung. Am J Respir Cell Mol Biol 18: 60-65. (Pubitemid 128355347)
91. Hashimoto N, Phan SH, Imaizumi K, Matsuo M, Nakashima H, Kawabe T, et al,. (2010). Endothelial-mesenchymal transition in bleomycin-induced pulmonary fibrosis. Am J Respir Cell Mol Biol 43: 161-172.
92. Haslett PA, Corral LG, Albert M, Kaplan G, (1998). Thalidomide costimulates primary human T lymphocytes, preferentially inducing proliferation, cytokine production, and cytotoxic responses in the CD8+ subset. J Exp Med 187: 1885-1892. (Pubitemid 28262451)
93. Hayakawa Y, Tazawa S, Ishikawa T, Niiya K, Sakuragawa N, (1995). Transcriptional regulation of tissue- and urokinase-type plasminogen activator genes by thrombin in human fetal lung fibroblasts. Thromb Haemost 74: 704-710.
94. Hecker L, Vittal R, Jones T, Jagirdar R, Luckhardt TR, Horowitz JC, et al,. (2009). NADPH oxidase-4 mediates myofibroblast activation and fibrogenic responses to lung injury. Nat Med 15: 1077-1081.
95. Henderson WR Jr, Chi EY, Ye X, Nguyen C, Tien YT, Zhou B, et al,. (2010). Inhibition of Wnt/beta-catenin/CREB binding protein (CBP) signaling reverses pulmonary fibrosis. Proc Natl Acad Sci USA 107: 14309-14314.
96. Hernandez-Rodriguez NA, Cambrey AD, Harrison NK, Chambers RC, Gray AJ, Southcott AM, et al,. (1995). Role of thrombin in pulmonary fibrosis. Lancet 346: 1071-1073.
97. Hewitson TD, Kelynack KJ, Tait MG, Martic M, Jones CL, Margolin SB, et al,. (2001). Pirfenidone reduces in vitro rat renal fibroblast activation and mitogenesis. J Nephrol 14: 453-460. (Pubitemid 34168279)
98. Higashiyama H, Yoshimoto D, Kaise T, Matsubara S, Fujiwara M, Kikkawa H, et al,. (2007). Inhibition of activin receptor-like kinase 5 attenuates bleomycin-induced pulmonary fibrosis. Exp Mol Pathol 83: 39-46. (Pubitemid 46842964)
99. Hogaboam CM, Lukacs NW, Chensue SW, Strieter RM, Kunkel SL, (1998). Monocyte chemoattractant protein-1 synthesis by murine lung fibroblasts modulates CD4+ T cell activation. J Immunol 160: 4606-4614. (Pubitemid 28217321)
100. Horan GS, Wood S, Ona V, Li DJ, Lukashev ME, Weinreb PH, et al,. (2008). Partial inhibition of integrin alpha(v)beta6 prevents pulmonary fibrosis without exacerbating inflammation. Am J Respir Crit Care Med 177: 56-65.
101. Howell DCJ, Goldsack NR, Marshall RP, McAnulty RJ, Starke R, Purdy G, et al,. (2001). Direct thrombin inhibition reduces lung collagen, accumulation, and connective tissue growth factor mrna levels in bleomycin-induced pulmonary fibrosis. Am J Pathol 159: 1383-1395. (Pubitemid 32947968)
102. Howell DCJ, Johns RH, Lasky JA, Shan B, Scotton CJ, Laurent GJ, et al,. (2005). Absence of proteinase-activated receptor-1 signaling affords protection from bleomycin-induced lung inflammation and fibrosis. Am J Pathol 166: 1353-1365. (Pubitemid 40586507)
103. Humphreys BD, Lin SL, Kobayashi A, Hudson TE, Nowlin BT, Bonventre JV, et al,. (2010). Fate tracing reveals the pericyte and not epithelial origin of myofibroblasts in kidney fibrosis. Am J Pathol 176: 85-97.
104. Imokawa S, Sato A, Hayakawa H, Kotani M, Urano T, Takada A, (1997). Tissue factor expression and fibrin deposition in the lungs of patients with idiopathic pulmonary fibrosis and systemic sclerosis. Am J Respir Crit Care Med 156: 631-636. (Pubitemid 27352005)
105. Iyer SN, Wild JS, Schiedt MJ, Hyde DM, Margolin SB, Giri SN, (1995). Dietary intake of pirfenidone ameliorates bleomycin-induced lung fibrosis in hamsters. J Lab Clin Med 125: 779-785.
106. Iyer SN, Gurujeyalakshmi G, Giri SN, (1999). Effects of pirfenidone on transforming growth factor-beta gene expression at the transcriptional level in bleomycin hamster model of lung fibrosis. J Pharmacol Exp Ther 291: 367-373. (Pubitemid 29451612)
107. Iyer SN, Hyde DM, Giri SN, (2000). Anti-inflammatory effect of pirfenidone in the bleomycin-hamster model of lung inflammation. Inflammation 24: 477-491. (Pubitemid 30452555)
108. Jack CI, Jackson MJ, Johnston ID, Hind CR, (1996). Serum indicators of free radical activity in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 153: 1918-1923. (Pubitemid 26187921)
109. Jakubzick C, Choi ES, Joshi BH, Keane MP, Kunkel SL, Puri RK, et al,. (2003). Therapeutic attenuation of pulmonary fibrosis via targeting of IL-4- and IL-13-responsive cells. J Immunol 171: 2684-2693. (Pubitemid 37025582)
110. Jenkins RG, Su X, Su G, Scotton CJ, Camerer E, Laurent GJ, et al,. (2006). Ligation of protease-activated receptor 1 enhances alpha(v)beta6 integrin-dependent TGF-beta activation and promotes acute lung injury. J Clin Invest 116: 1606-1614.
111. Kagan HM, Li W, (2003). Lysyl oxidase: properties, specificity, and biological roles inside and outside of the cell. J Cell Biochem 88: 660-672. (Pubitemid 36258816)
112. Kakugawa T, Mukae H, Hayashi T, Ishii H, Abe K, Fujii T, et al,. (2004). Pirfenidone attenuates expression of HSP47 in murine bleomycin-induced pulmonary fibrosis. Eur Respir J 24: 57-65. (Pubitemid 39029069)
113. Kaminski N, Rosas IO, (2006). Gene expression profiling as a window into idiopathic pulmonary fibrosis pathogenesis: can we identify the right target genes? Proc Am Thorac Soc 3: 339-344. (Pubitemid 43852873)
114. Karpus WJ, Lukacs NW, Kennedy KJ, Smith WS, Hurst SD, Barrett TA, (1997). Differential CC chemokine-induced enhancement of T helper cell cytokine production. J Immunol 158: 4129-4136. (Pubitemid 127483911)
115. Kasper M, Haroske G, (1996). Alterations in the alveolar epithelium after injury leading to pulmonary fibrosis. Histol Histopathol 11: 463-483. (Pubitemid 26135211)
116. Katzenstein AL, (1985). Pathogenesis of 'fibrosis' in interstitial pneumonia: an electron microscopic study. Hum Pathol 16: 1015-1024.
117. Katzenstein AL, Myers JL, (1998). Idiopathic pulmonary fibrosis: clinical relevance of pathologic classification. Am J Respir Crit Care Med 157: 1301-1315.
118. Kawanami O, Ferrans VJ, Crystal RG, (1982). Structure of alveolar epithelial cells in patients with fibrotic lung disorders. Lab Invest 46: 39-53. (Pubitemid 12167015)
119. Keane MP, Arenberg DA, Lynch JP 3rd, Whyte RI, Iannettoni MD, Burdick MD, et al,. (1997). The CXC chemokines, IL-8 and IP-10, regulate angiogenic activity in idiopathic pulmonary fibrosis. J Immunol 159: 1437-1443. (Pubitemid 127484123)
120. Keane MP, Belperio JA, Arenberg DA, Burdick MD, Xu ZJ, Xue YY, et al,. (1999). IFN-gamma-inducible protein-10 attenuates bleomycin-induced pulmonary fibrosis via inhibition of angiogenesis. J Immunol 163: 5686-5692.
121. Keane MP, Belperio JA, Burdick MD, Lynch JP, Fishbein MC, Strieter RM, (2001). ENA-78 is an important angiogenic factor in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 164: 2239-2242. (Pubitemid 34032941)
122. Keerthisingam CB, Jenkins RG, Harrison NK, Hernandez-Rodriguez NA, Booth H, Laurent GJ, et al,. (2001). Cyclooxygenase-2 deficiency results in a loss of the anti-proliferative response to transforming growth factor-beta in human fibrotic lung fibroblasts and promotes bleomycin-induced pulmonary fibrosis in mice. Am J Pathol 158: 1411-1422. (Pubitemid 32281305)
123. Kelly BG, Lok SS, Hasleton PS, Egan JJ, Stewart JP, (2002). A rearranged form of Epstein-Barr virus DNA is associated with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 166: 510-513.
124. Khalil N, O'Connor RN, Flanders KC, Unruh H, (1996). TGF-beta 1, but not TGF-beta 2 or TGF-beta 3, is differentially present in epithelial cells of advanced pulmonary fibrosis: an immunohistochemical study. Am J Respir Cell Mol Biol 14: 131-138. (Pubitemid 126441385)
125. Kikuchi A, Yamamoto H, Kishida S, (2007). Multiplicity of the interactions of Wnt proteins and their receptors. Cell Signal 19: 659-671. (Pubitemid 46275377)
126. Kim KK, Kugler MC, Wolters PJ, Robillard L, Galvez MG, Brumwell AN, et al,. (2006). Alveolar epithelial cell mesenchymal transition develops in vivo during pulmonary fibrosis and is regulated by the extracellular matrix. Proc Natl Acad Sci USA 103: 13180-13185. (Pubitemid 44338936)
127. Kim KK, Wei Y, Szekeres C, Kugler MC, Wolters PJ, Hill ML, et al,. (2009). Epithelial cell alpha3beta1 integrin links beta-catenin and Smad signaling to promote myofibroblast formation and pulmonary fibrosis. J Clin Invest 119: 213-224.
128. King TE Jr, Tooze JA, Schwarz MI, Brown KR, Cherniack RM, (2001). Predicting survival in idiopathic pulmonary fibrosis: scoring system and survival model. Am J Respir Crit Care Med 164: 1171-1181. (Pubitemid 33052246)
129. King TE Jr, Behr J, Brown KK, du Bois RM, Lancaster L, de Andrade JA, et al,. (2008). BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 177: 75-81.
130. King TE Jr, Albera C, Bradford WZ, Costabel U, Hormel P, Lancaster L, et al,. (2009). Effect of interferon gamma-1b on survival in patients with idiopathic pulmonary fibrosis (INSPIRE): a multicentre, randomised, placebo-controlled trial. Lancet 374: 222-228.
131. Koch HP, (1985). Thalidomide and congeners as anti-inflammatory agents. Prog Med Chem 22: 165-242.
132. Kolb M, Kirschner J, Riedel W, Wirtz H, Schmidt M, (1998). Cyclophosphamide pulse therapy in idiopathic pulmonary fibrosis. Eur Respir J 12: 1409-1414. (Pubitemid 29011438)
133. Kollias G, Douni E, Kassiotis G, Kontoyiannis D, (1999). On the role of tumor necrosis factor and receptors in models of multiorgan failure, rheumatoid arthritis, multiple sclerosis and inflammatory bowel disease. Immunol Rev 169: 175-194. (Pubitemid 29338732)
134. Kolodsick JE, Peters-Golden M, Larios J, Toews GB, Thannickal VJ, Moore BB, (2003). Prostaglandin E2 inhibits fibroblast to myofibroblast transition via E. prostanoid receptor 2 signaling and cyclic adenosine monophosphate elevation. Am J Respir Cell Mol Biol 29: 537-544. (Pubitemid 37357704)
135. Kolodsick JE, Toews GB, Jakubzick C, Hogaboam C, Moore TA, McKenzie A, et al,. (2004). Protection from fluorescein isothiocyanate-induced fibrosis in IL-13-deficient, but not IL-4-deficient, mice results from impaired collagen synthesis by fibroblasts. J Immunol 172: 4068-4076. (Pubitemid 38375218)
136. Komuro I, Kurihara H, Sugiyama T, Yoshizumi M, Takaku F, Yazaki Y, (1988). Endothelin stimulates c-fos and c-myc expression and proliferation of vascular smooth muscle cells. FEBS Lett 238: 249-252.
137. Kondoh Y, Taniguchi H, Yokoi T, Nishiyama O, Ohishi T, Kato T, et al,. (2005). Cyclophosphamide and low-dose prednisolone in idiopathic pulmonary fibrosis and fibrosing nonspecific interstitial pneumonia. Eur Respir J 25: 528-533. (Pubitemid 40331185)
138. Konigshoff M, Kramer M, Balsara N, Wilhelm J, Amarie OV, Jahn A, et al,. (2009). WNT1-inducible signaling protein-1 mediates pulmonary fibrosis in mice and is upregulated in humans with idiopathic pulmonary fibrosis. J Clin Invest 119: 772-787.
139. Korfei M, Ruppert C, Mahavadi P, Henneke I, Markart P, Koch M, et al,. (2008). Epithelial endoplasmic reticulum stress and apoptosis in sporadic idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 178: 838-846.
140. Krowka MJ, Ahmad S, de Andrade JA, Frost A, Glassberg M, Lancaster L, et al,. (2007). A ranomized, double-blind trial, placebo-controlled study to evaluate the safety and efficacy of iloprost inhalation in adults with abnormal pulmonary arterial pressure and exercise limitation associated with idiopathic pulmonary fibrosis. Chest (Suppl) 132: 633a.
141. Kubo H, Nakayama K, Yanai M, Suzuki T, Yamaya M, Watanabe M, et al,. (2005). Anticoagulant therapy for idiopathic pulmonary fibrosis. Chest 128: 1475-1482. (Pubitemid 41324435)
142. Kuhn C, McDonald JA, (1991). The roles of the myofibroblast in idiopathic pulmonary fibrosis. Ultrastructural and immunohistochemical features of sites of active extracellular matrix synthesis. Am J Pathol 138: 1257-1265.
143. Kuwano K, Kunitake R, Kawasaki M, Nomoto Y, Hagimoto N, Nakanishi Y, et al,. (1996). P21Waf1/Cip1/Sdi1 and p53 expression in association with DNA strand breaks in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 154: 477-483. (Pubitemid 26269122)
144. Lama V, Moore BB, Christensen P, Toews GB, Peters-Golden M, (2002). Prostaglandin E2 synthesis and suppression of fibroblast proliferation by alveolar epithelial cells is cyclooxygenase-2-dependent. Am J Respir Cell Mol Biol 27: 752-758. (Pubitemid 35381185)
145. Lama VN, Phan SH, (2006). The extrapulmonary origin of fibroblasts: stem/progenitor cells and beyond. Proc Am Thorac Soc 3: 373-376. (Pubitemid 43845415)
146. Latsi PI, du Bois RM, Nicholson AG, Colby TV, Bisirtzoglou D, Nikolakopoulou A, et al,. (2003). Fibrotic idiopathic interstitial pneumonia: the prognostic value of longitudinal functional trends. Am J Respir Crit Care Med 168: 531-537. (Pubitemid 37088594)
147. Lawson WE, Grant SW, Ambrosini V, Womble KE, Dawson EP, Lane KB, et al,. (2004). Genetic mutations in surfactant protein C are a rare cause of sporadic cases of IPF. Thorax 59: 977-980. (Pubitemid 39490784)
148. Lawson WE, Crossno PF, Polosukhin VV, Roldan J, Cheng DS, Lane KB, et al,. (2008). Endoplasmic reticulum stress in alveolar epithelial cells is prominent in IPF: association with altered surfactant protein processing and herpesvirus infection. Am J Physiol Lung Cell Mol Physiol 294: L1119-L1126.
149. Leask A, (2009). Signaling in fibrosis: targeting the TGF beta, endothelin-1 and CCN2 axis in scleroderma. Front Biosci (Elite Ed) 1: 115-122.
150. Leask A, Abraham DJ, (2003). The role of connective tissue growth factor, a multifunctional matricellular protein, in fibroblast biology. Biochem Cell Biol 81: 355-363. (Pubitemid 38186975)
151. Lee CG, Cho SJ, Kang MJ, Chapoval SP, Lee PJ, Noble PW, et al,. (2004). Early growth response gene 1-mediated apoptosis is essential for transforming growth factor beta1-induced pulmonary fibrosis. J Exp Med 200: 377-389. (Pubitemid 39031256)
152. Lepisto J, Peltonen J, Vaha-Kreula M, Niinikoski J, Laato M, (1995). Platelet-derived growth factor isoforms PDGF-AA, -AB and -BB exert specific effects on collagen gene expression and mitotic activity of cultured human wound fibroblasts. Biochem Biophys Res Commun 209: 393-399.
153. Li X, Rayford H, Uhal BD, (2003a). Essential roles for angiotensin receptor AT1a in bleomycin-induced apoptosis and lung fibrosis in mice. Am J Pathol 163: 2523-2530. (Pubitemid 37466493)
154. Li X, Zhang H, Soledad-Conrad V, Zhuang J, Uhal BD, (2003b). Bleomycin-induced apoptosis of alveolar epithelial cells requires angiotensin synthesis de novo. Am J Physiol Lung Cell Mol Physiol 284: L501-L507. (Pubitemid 36231728)
155. Li X, Molina-Molina M, Abdul-Hafez A, Ramirez J, Serrano-Mollar A, Xaubet A, et al,. (2006). Extravascular sources of lung angiotensin peptide synthesis in idiopathic pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol 291: L887-L895. (Pubitemid 44772050)
156. Liu T, Jin H, Ullenbruch M, Hu B, Hashimoto N, Moore B, et al,. (2004). Regulation of found in inflammatory zone 1 expression in bleomycin-induced lung fibrosis: role of IL-4/IL-13 and mediation via STAT-6. J Immunol 173: 3425-3431. (Pubitemid 39142053)
157. Liu G, Friggeri A, Yang Y, Milosevic J, Ding Q, Thannickal VJ, et al,. (2010a). miR-21 mediates fibrogenic activation of pulmonary fibroblasts and lung fibrosis. J Exp Med 207: 1589-1597.
158. Liu S, Taghavi R, Leask A, (2010b). Connective tissue growth factor is induced in bleomycin-induced skin scleroderma. J Cell Commun Signal 4: 25-30.
159. Lovgren AK, Jania LA, Hartney JM, Parsons KK, Audoly LP, Fitzgerald GA, et al,. (2006). COX-2-derived prostacyclin protects against bleomycin-induced pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol 291: L144-L156. (Pubitemid 44148098)
160. McAnulty RJ, Campa JS, Cambrey AD, Laurent GJ, (1991). The effect of transforming growth factor beta on rates of procollagen synthesis and degradation in vitro. Biochim Biophys Acta 1091: 231-235.
161. MacNee W, Rahman I, (1995). Oxidants/antioxidants in idiopathic pulmonary fibrosis. Thorax 50 (Suppl. 1): S53-S58.
162. Maeyama T, Kuwano K, Kawasaki M, Kunitake R, Hagimoto N, Matsuba T, et al,. (2001). Upregulation of Fas-signalling molecules in lung epithelial cells from patients with idiopathic pulmonary fibrosis. Eur Respir J 17: 180-189. (Pubitemid 32600575)
163. Maher TM, (2010). Pirfenidone in idiopathic pulmonary fibrosis. Drugs Today (Barc) 46: 473-482.
164. Maher TM, Evans IC, Bottoms SE, Mercer PF, Thorley AJ, Nicholson AG, et al,. (2010a). Diminished prostaglandin E2 contributes to the apoptosis paradox in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 182: 73-82.
165. Maher TM, Evans IC, Laurent GJ, McAnulty RJ, (2010b). PGE2 paradoxically increases fibroblast apoptosis but reduces airway epithelial cell apoptosis in response to FasL via activation of the EP4 receptor. Am J Respir Crit Care Med 181 (Suppl.): A4182.
166. Marchal-Somme J, Uzunhan Y, Marchand-Adam S, Kambouchner M, Valeyre D, Crestani B, et al,. (2007). Dendritic cells accumulate in human fibrotic interstitial lung disease. Am J Respir Crit Care Med 176: 1007-1014. (Pubitemid 350127845)
167. Markart P, Luboeinski T, Korfei M, Schmidt R, Wygrecka M, Mahavadi P, et al,. (2009). Alveolar oxidative stress is associated with elevated levels of nonenzymatic low-molecular-weight antioxidants in patients with different forms of chronic fibrosing interstitial lung diseases. Antioxid Redox Signal 11: 227-240.
168. Markart P, Nass R, Ruppert C, Hundack L, Wygrecka M, Korfei M, et al,. (2010). Safety and tolerability of inhaled heparin in idiopathic pulmonary fibrosis. J Aerosol Med Pulm Drug Deliv 23: 161-172.
169. Marshall RP, Webb S, Bellingan GJ, Montgomery HE, Chaudhari B, McAnulty RJ, et al,. (2002). Angiotensin converting enzyme insertion/deletion polymorphism is associated with susceptibility and outcome in acute respiratory distress syndrome. Am J Respir Crit Care Med 166: 646-650. (Pubitemid 34971844)
170. Marshall RP, Gohlke P, Chambers RC, Howell DC, Bottoms SE, Unger T, et al,. (2004). Angiotensin II and the fibroproliferative response to acute lung injury. Am J Physiol Lung Cell Mol Physiol 286: L156-L164. (Pubitemid 38055871)
171. Mehrad B, Burdick MD, Strieter RM, (2009). Fibrocyte CXCR4 regulation as a therapeutic target in pulmonary fibrosis. Int J Biochem Cell Biol 41: 1708-1718.
172. Miyazaki Y, Araki K, Vesin C, Garcia I, Kapanci Y, Whitsett JA, et al,. (1995). Expression of a tumor necrosis factor-alpha transgene in murine lung causes lymphocytic and fibrosing alveolitis. A mouse model of progressive pulmonary fibrosis. J Clin Invest 96: 250-259.
173. Mizuno S, Matsumoto K, Li MY, Nakamura T, (2005). HGF reduces advancing lung fibrosis in mice: a potential role for MMP-dependent myofibroblast apoptosis. FASEB J 19: 580-582. (Pubitemid 40471250)
174. Moeller A, Ask K, Warburton D, Gauldie J, Kolb M, (2008). The bleomycin animal model: a useful tool to investigate treatment options for idiopathic pulmonary fibrosis? Int J Biochem Cell Biol 40: 362-382. (Pubitemid 351249887)
175. Moeller A, Gilpin SE, Ask K, Cox G, Cook D, Gauldie J, et al,. (2009). Circulating fibrocytes are an indicator of poor prognosis in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 179: 588-594.
176. Montaldo C, Cannas E, Ledda M, Rosetti L, Congiu L, Atzori L, (2002). Bronchoalveolar glutathione and nitrite/nitrate in idiopathic pulmonary fibrosis and sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 19: 54-58. (Pubitemid 34298495)
177. Montuschi P, Ciabattoni G, Paredi P, Pantelidis P, du Bois RM, Kharitonov SA, et al,. (1998). 8-Isoprostane as a biomarker of oxidative stress in interstitial lung diseases. Am J Respir Crit Care Med 158: 1524-1527.
178. Moodley YP, Scaffidi AK, Misso NL, Keerthisingam C, McAnulty RJ, Laurent GJ, et al,. (2003). Fibroblasts isolated from normal lungs and those with idiopathic pulmonary fibrosis differ in interleukin-6/gp130-mediated cell signaling and proliferation. Am J Pathol 163: 345-354. (Pubitemid 36759619)
179. Moodley YP, Caterina P, Scaffidi AK, Misso NL, Papadimitriou JM, McAnulty RJ, et al,. (2004). Comparison of the morphological and biochemical changes in normal human lung fibroblasts and fibroblasts derived from lungs of patients with idiopathic pulmonary fibrosis during FasL-induced apoptosis. J Pathol 202: 486-495. (Pubitemid 38456167)
180. Moore BB, Kolodsick JE, Thannickal VJ, Cooke K, Moore TA, Hogaboam C, et al,. (2005). CCR2-mediated recruitment of fibrocytes to the alveolar space after fibrotic injury. Am J Pathol 166: 675-684. (Pubitemid 40315625)
181. Moore BB, Murray L, Das A, Wilke CA, Herrygers AB, Toews GB, (2006). The role of CCL12 in the recruitment of fibrocytes and lung fibrosis. Am J Respir Cell Mol Biol 35: 175-181. (Pubitemid 44175316)
182. Munger JS, Huang X, Kawakatsu H, Griffiths MJ, Dalton SL, Wu J, et al,. (1999). The integrin alpha v beta 6 binds and activates latent TGF beta 1: a mechanism for regulating pulmonary inflammation and fibrosis. Cell 96: 319-328. (Pubitemid 29077586)
183. Murphy KM, Ouyang W, Farrar JD, Yang J, Ranganath S, Asnagli H, et al,. (2000). Signaling and transcription in T helper development. Annu Rev Immunol 18: 451-494. (Pubitemid 30365388)
184. Murphy-Ullrich JE, Poczatek M, (2000). Activation of latent TGF-beta by thrombospondin-1: mechanisms and physiology. Cytokine Growth Factor Rev 11: 59-69. (Pubitemid 30169726)
185. Mutsaers SE, Marshall RP, Goldsack NR, Laurent GJ, McAnulty RJ, (1998). Effect of endothelin receptor antagonists (BQ-485, Ro 47-0203) on collagen deposition during the development of bleomycin-induced pulmonary fibrosis in rats. Pulm Pharmacol Ther 11: 221-225. (Pubitemid 29041586)
186. Nakazato H, Oku H, Yamane S, Tsuruta Y, Suzuki R, (2002). A novel anti-fibrotic agent pirfenidone suppresses tumor necrosis factor-alpha at the translational level. Eur J Pharmacol 446: 177-185. (Pubitemid 34722572)
187. Nash JR, McLaughlin PJ, Butcher D, Corrin B, (1993). Expression of tumour necrosis factor-alpha in cryptogenic fibrosing alveolitis. Histopathology 22: 343-347. (Pubitemid 23159794)
188. Nyhan KC, Faherty N, Murray G, Cooey LB, Godson C, Crean JK, et al,. (2010). Jagged/Notch signalling is required for a subset of TGFbeta1 responses in human kidney epithelial cells. Biochim Biophys Acta 1803: 1386-1395.
189. Oikonomou N, Harokopos V, Zalevsky J, Valavanis C, Kotanidou A, Szymkowski DE, et al,. (2006). Soluble TNF mediates the transition from pulmonary inflammation to fibrosis. PloS ONE 1: e108.
190. Oku H, Nakazato H, Horikawa T, Tsuruta Y, Suzuki R, (2002). Pirfenidone suppresses tumor necrosis factor-alpha, enhances interleukin-10 and protects mice from endotoxic shock. Eur J Pharmacol 446: 167-176. (Pubitemid 34722571)
191. Oriente A, Fedarko NS, Pacocha SE, Huang SK, Lichtenstein LM, Essayan DM, (2000). Interleukin-13 modulates collagen homeostasis in human skin and keloid fibroblasts. J Pharmacol Exp Ther 292: 988-994. (Pubitemid 30136592)
192. Ortiz LA, Lasky J, Hamilton RF Jr, Holian A, Hoyle GW, Banks W, et al,. (1998). Expression of TNF and the necessity of TNF receptors in bleomycin-induced lung injury in mice. Exp Lung Res 24: 721-743. (Pubitemid 28533206)
193. Otsuka M, Takahashi H, Shiratori M, Chiba H, Abe S, (2004). Reduction of bleomycin induced lung fibrosis by candesartan cilexetil, an angiotensin II type 1 receptor antagonist. Thorax 59: 31-38. (Pubitemid 38067969)
194. Overall CM, Wrana JL, Sodek J, (1989). Transforming growth factor-beta regulation of collagenase, 72 kDa-progelatinase, TIMP and PAI-1 expression in rat bone cell populations and human fibroblasts. Connect Tissue Res 20: 289-294.
195. Ozawa Y, Suda T, Naito T, Enomoto N, Hashimoto D, Fujisawa T, et al,. (2009). Cumulative incidence of and predictive factors for lung cancer in IPF. Respirology 14: 723-728.
196. Pan Q, Kleer CG, van Golen KL, Irani J, Bottema KM, Bias C, et al,. (2002). Copper deficiency induced by tetrathiomolybdate suppresses tumor growth and angiogenesis. Cancer Res 62: 4854-4859. (Pubitemid 34984403)
197. Pandit KV, Corcoran D, Yousef H, Yarlagadda M, Tzouvelekis A, Gibson KF, et al,. (2010). Inhibition and role of let-7d in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 182: 220-229.
198. Park SH, Saleh D, Giaid A, Michel RP, (1997). Increased endothelin-1 in bleomycin-induced pulmonary fibrosis and the effect of an endothelin receptor antagonist. Am J Respir Crit Care Med 156: 600-608. (Pubitemid 27352001)
199. Peacock AJ, Dawes KE, Shock A, Gray AJ, Reeves JT, Laurent GJ, (1992). Endothelin-1 and endothelin-3 induce chemotaxis and replication of pulmonary artery fibroblasts. Am J Respir Cell Mol Biol 7: 492-499.
200. Peao MN, Aguas AP, de Sa CM, Grande NR, (1994). Neoformation of blood vessels in association with rat lung fibrosis induced by bleomycin. Anat Rec 238: 57-67. (Pubitemid 24002449)
201. Pedram A, Razandi M, Hu RM, Levin ER, (1997). Vasoactive peptides modulate vascular endothelial cell growth factor production and endothelial cell proliferation and invasion. J Biol Chem 272: 17097-17103. (Pubitemid 27289818)
202. Peters-Golden M, Bailie M, Marshall T, Wilke C, Phan SH, Toews GB, et al,. (2002). Protection from pulmonary fibrosis in leukotriene-deficient mice. Am J Respir Crit Care Med 165: 229-235. (Pubitemid 34084463)
203. Phan SH, (2002). The myofibroblast in pulmonary fibrosis. Chest 122: 286S-289S. (Pubitemid 36043014)
204. Phelps DT, Deneke SM, Daley DL, Fanburg BL, (1992). Elevation of glutathione levels in bovine pulmonary artery endothelial cells by N-acetylcysteine. Am J Respir Cell Mol Biol 7: 293-299.
205. Phillips RJ, Burdick MD, Hong K, Lutz MA, Murray LA, Xue YY, et al,. (2004). Circulating fibrocytes traffic to the lungs in response to CXCL12 and mediate fibrosis. J Clin Invest 114: 438-446. (Pubitemid 39071616)
206. Piguet PF, Collart MA, Grau GE, Kapanci Y, Vassalli P, (1989). Tumor necrosis factor/cachectin plays a key role in bleomycin-induced pneumopathy and fibrosis. J Exp Med 170: 655-663. (Pubitemid 19226654)
207. Piguet PF, Ribaux C, Karpuz V, Grau GE, Kapanci Y, (1993). Expression and localization of tumor necrosis factor-alpha and its mRNA in idiopathic pulmonary fibrosis. Am J Pathol 143: 651-655. (Pubitemid 24058221)
208. Plataki M, Koutsopoulos AV, Darivianaki K, Delides G, Siafakas NM, Bouros D, (2005). Expression of apoptotic and antiapoptotic markers in epithelial cells in idiopathic pulmonary fibrosis. Chest 127: 266-274. (Pubitemid 41724587)
209. Raghow R, Postlethwaite AE, Keski-Oja J, Moses HL, Kang AH, (1987). Transforming growth factor-beta increases steady state levels of type I procollagen and fibronectin messenger RNAs posttranscriptionally in cultured human dermal fibroblasts. J Clin Invest 79: 1285-1288. (Pubitemid 17065515)
210. Raghu G, Depaso WJ, Cain K, Hammar SP, Wetzel CE, Dreis DF, et al,. (1991). Azathioprine combined with prednisone in the treatment of idiopathic pulmonary fibrosis: a prospective double-blind, randomized, placebo-controlled clinical trial. Am Rev Respir Dis 144: 291-296.
211. Raghu G, Brown KK, Bradford WZ, Starko K, Noble PW, Schwartz DA, et al,. (2004). A placebo-controlled trial of interferon gamma-1b in patients with idiopathic pulmonary fibrosis. N Engl J Med 350: 125-133. (Pubitemid 38056223)
212. Raghu G, Brown KK, Costabel U, Cottin V, du Bois RM, Lasky JA, et al,. (2008). Treatment of idiopathic pulmonary fibrosis with etanercept: an exploratory, placebo-controlled trial. Am J Respir Crit Care Med 178: 948-955.
213. Rahman I, Skwarska E, Henry M, Davis M, O'Connor CM, FitzGerald MX, et al,. (1999). Systemic and pulmonary oxidative stress in idiopathic pulmonary fibrosis. Free Radic Biol Med 27: 60-68. (Pubitemid 29353084)
214. Ramos C, Montano M, Garcia-Alvarez J, Ruiz V, Uhal BD, Selman M, et al,. (2001). Fibroblasts from idiopathic pulmonary fibrosis and normal lungs differ in growth rate, apoptosis, and tissue inhibitor of metalloproteinases expression. Am J Respir Cell Mol Biol 24: 591-598. (Pubitemid 32442767)
215. Reddy NM, Kleeberger SR, Cho HY, Yamamoto M, Kensler TW, Biswal S, et al,. (2007). Deficiency in Nrf2-GSH signaling impairs type II cell growth and enhances sensitivity to oxidants. Am J Respir Cell Mol Biol 37: 3-8. (Pubitemid 46999531)
216. Renzoni EA, (2004). Neovascularization in idiopathic pulmonary fibrosis: too much or too little? Am J Respir Crit Care Med 169: 1179-1180. (Pubitemid 38680392)
217. Renzoni EA, Walsh DA, Salmon M, Wells AU, Sestini P, Nicholson AG, et al,. (2003). Interstitial vascularity in fibrosing alveolitis. Am J Respir Crit Care Med 167: 438-443. (Pubitemid 36216429)
218. Rose CE Jr, Sung SS, Fu SM, (2003). Significant involvement of CCL2 (MCP-1) in inflammatory disorders of the lung. Microcirculation 10: 273-288. (Pubitemid 39004255)
219. Rosenbloom J, Feldman G, Freundlich B, Jimenez SA, (1986). Inhibition of excessive scleroderma fibroblast collagen production by recombinant gamma-interferon. Association with a coordinate decrease in types I and III procollagen messenger RNA levels. Arthritis Rheum 29: 851-856. (Pubitemid 16072883)
220. Rubin JS, Osada H, Finch PW, Taylor WG, Rudikoff S, Aaronson SA, (1989). Purification and characterization of a newly identified growth factor specific for epithelial cells. Proc Natl Acad Sci USA 86: 802-806. (Pubitemid 19056244)
221. Saito A, Okazaki H, Sugawara I, Yamamoto K, Takizawa H, (2003). Potential action of IL-4 and IL-13 as fibrogenic factors on lung fibroblasts in vitro. Int Arch Allergy Immunol 132: 168-176. (Pubitemid 37409848)
222. Saleh D, Barnes PJ, Giaid A, (1997a). Increased production of the potent oxidant peroxynitrite in the lungs of patients with idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 155: 1763-1769. (Pubitemid 27216690)
223. Saleh D, Furukawa K, Tsao MS, Maghazachi A, Corrin B, Yanagisawa M, et al,. (1997b). Elevated expression of endothelin-1 and endothelin-converting enzyme-1 in idiopathic pulmonary fibrosis: possible involvement of proinflammatory cytokines. Am J Respir Cell Mol Biol 16: 187-193. (Pubitemid 127498240)
224. Schlingensiepen KH, Fischer-Blass B, Schmaus S, Ludwig S, (2008). Antisense therapeutics for tumor treatment: the TGF-beta2 inhibitor AP 12009 in clinical development against malignant tumors. Recent Results Cancer Res 177: 137-150.
225. Scotton CJ, Chambers RC, (2007). Molecular targets in pulmonary fibrosis: the myofibroblast in focus. Chest 132: 1311-1321. (Pubitemid 47621009)
226. Scotton CJ, Chambers RC, (2010). Bleomycin revisited: towards a more representative model of IPF? Am J Physiol Lung Cell Mol Physiol 299: L439-L441.
227. Scotton CJ, Krupiczojc MA, Konigshoff M, Mercer PF, Lee YC, Kaminski N, et al,. (2009). Increased local expression of coagulation factor X contributes to the fibrotic response in human and murine lung injury. J Clin Invest 119: 2550-2563.
228. Selman M, Pardo A, (2002). Idiopathic pulmonary fibrosis: an epithelial/fibroblastic cross-talk disorder. Respir Res 3: 3.
229. Selman M, Carrillo G, Salas J, Padilla RP, Perez-Chavira R, Sansores R, et al,. (1998). Colchicine, d-penicillamine, and prednisone in the treatment of idiopathic pulmonary fibrosis: a controlled clinical trial. Chest 114: 507-512. (Pubitemid 28387531)
230. Shah M, Foreman DM, Ferguson MW, (1995). Neutralisation of TGF-beta 1 and TGF-beta 2 or exogenous addition of TGF-beta 3 to cutaneous rat wounds reduces scarring. J Cell Sci 108: 985-1002.
231. Shahar I, Fireman E, Topilsky M, Grief J, Schwarz Y, Kivity S, et al,. (1999). Effect of endothelin-1 on alpha-smooth muscle actin expression and on alveolar fibroblasts proliferation in interstitial lung diseases. Int J Immunopharmacol 21: 759-775. (Pubitemid 29472503)
232. Shahzeidi S, Sarnstrand B, Jeffery PK, McAnulty RJ, Laurent GJ, (1991). Oral N-acetylcysteine reduces bleomycin-induced collagen deposition in the lungs of mice. Eur Respir J 4: 845-852.
233. Shi-Wen X, Chen Y, Denton CP, Eastwood M, Renzoni EA, Bou-Gharios G, et al,. (2004). Endothelin-1 promotes myofibroblast induction through the ETA receptor via a rac/phosphoinositide 3-kinase/Akt-dependent pathway and is essential for the enhanced contractile phenotype of fibrotic fibroblasts. Mol Biol Cell 15: 2707-2719. (Pubitemid 38691860)
234. Sime PJ, Xing Z, Graham FL, Csaky KG, Gauldie J, (1997). Adenovector-mediated gene transfer of active transforming growth factor-beta1 induces prolonged severe fibrosis in rat lung. J Clin Invest 100: 768-776. (Pubitemid 27371693)
235. Sime PJ, Marr RA, Gauldie D, Xing Z, Hewlett BR, Graham FL, et al,. (1998). Transfer of tumor necrosis factor-alpha to rat lung induces severe pulmonary inflammation and patchy interstitial fibrogenesis with induction of transforming growth factor-beta1 and myofibroblasts. Am J Pathol 153: 825-832. (Pubitemid 28419035)
236. Simler NR, Howell DC, Marshall RP, Goldsack NR, Hasleton PS, Laurent GJ, et al,. (2002). The rapamycin analogue SDZ RAD attenuates bleomycin-induced pulmonary fibrosis in rats. Eur Respir J 19: 1124-1127. (Pubitemid 34649629)
237. Sonnylal S, Shi-Wen X, Leoni P, Naff K, Van Pelt CS, Nakamura H, et al,. (2010). Selective expression of connective tissue growth factor in fibroblasts in vivo promotes systemic tissue fibrosis. Arthritis Rheum 62: 1523-1532.
238. Stefani G, Slack FJ, (2008). Small non-coding RNAs in animal development. Nat Rev Mol Cell Biol 9: 219-230. (Pubitemid 351301826)
239. Strieter RM, (2005). Pathogenesis and natural history of usual interstitial pneumonia: the whole story or the last chapter of a long novel. Chest 128: 526S-532S.
240. Subramanian SV, Polikandriotis JA, Kelm RJ Jr, David JJ, Orosz CG, Strauch AR, (2004). Induction of vascular smooth muscle alpha-actin gene transcription in transforming growth factor beta1-activated myofibroblasts mediated by dynamic interplay between the Pur repressor proteins and Sp1/Smad coactivators. Mol Biol Cell 15: 4532-4543. (Pubitemid 39299102)
241. Suga M, Iyonaga K, Ichiyasu H, Saita N, Yamasaki H, Ando M, (1999). Clinical significance of MCP-1 levels in BALF and serum in patients with interstitial lung diseases. Eur Respir J 14: 376-382. (Pubitemid 29461305)
242. Suganuma H, Sato A, Tamura R, Chida K, (1995). Enhanced migration of fibroblasts derived from lungs with fibrotic lesions. Thorax 50: 984-989.
243. Sutinen S, Rainio P, Sutinen S, Huhti E, Pokela R, (1980). Ultrastructure of terminal respiratory epithelium and prognosis in chronic interstitial pneumonia. Eur J Respir Dis 61: 325-336. (Pubitemid 11174764)
244. Tabata C, Tabata R, Kadokawa Y, Hisamori S, Takahashi M, Mishima M, et al,. (2007). Thalidomide prevents bleomycin-induced pulmonary fibrosis in mice. J Immunol 179: 708-714. (Pubitemid 46986590)
245. Tager AM, LaCamera P, Shea BS, Campanella GS, Selman M, Zhao Z, et al,. (2008). The lysophosphatidic acid receptor LPA1 links pulmonary fibrosis to lung injury by mediating fibroblast recruitment and vascular leak. Nat Med 14: 45-54.
246. Tamargo RJ, Bok RA, Brem H, (1991). Angiogenesis inhibition by minocycline. Cancer Res 51: 672-675.
247. Tang YW, Johnson JE, Browning PJ, Cruz-Gervis RA, Davis A, Graham BS, et al,. (2003). Herpesvirus DNA is consistently detected in lungs of patients with idiopathic pulmonary fibrosis. J Clin Microbiol 41: 2633-2640. (Pubitemid 36712478)
248. Taniguchi H, Ebina M, Kondoh Y, Ogura T, Azuma A, Suga M, et al,. (2010). Pirfenidone in idiopathic pulmonary fibrosis. Eur Respir J 35: 821-829.
249. Turner-Warwick M, (1963). Precapillary systemic-pulmonary anastomoses. Thorax 18: 225-237.
250. Turner-Warwick M, Burrows B, Johnson A, (1980). Cryptogenic fibrosing alveolitis: response to corticosteroid treatment and its effect on survival. Thorax 35: 593-599. (Pubitemid 10055764)
251. Uhal BD, Joshi I, Hughes WF, Ramos C, Pardo A, Selman M, (1998). Alveolar epithelial cell death adjacent to underlying myofibroblasts in advanced fibrotic human lung. Am J Physiol 275: L1192-L1199.
252. Uhal BD, Kim JK, Li X, Molina-Molina M, (2007). Angiotensin-TGF-beta 1 crosstalk in human idiopathic pulmonary fibrosis: autocrine mechanisms in myofibroblasts and macrophages. Curr Pharm Des 13: 1247-1256. (Pubitemid 46739332)
253. Ulloa L, Doody J, Massague J, (1999). Inhibition of transforming growth factor-beta/SMAD signalling by the interferon-gamma/STAT pathway. Nature 397: 710-713.
254. Waghray M, Cui Z, Horowitz JC, Subramanian IM, Martinez FJ, Toews GB, et al,. (2005). Hydrogen peroxide is a diffusible paracrine signal for the induction of epithelial cell death by activated myofibroblasts. FASEB J 19: 854-856. (Pubitemid 40617407)
255. Walters DM, Cho HY, Kleeberger SR, (2008). Oxidative stress and antioxidants in the pathogenesis of pulmonary fibrosis: a potential role for Nrf2. Antioxid Redox Signal 10: 321-332.
256. Wang R, Zagariya A, Ibarra-Sunga O, Gidea C, Ang E, Deshmukh S, et al,. (1999). Angiotensin II induces apoptosis in human and rat alveolar epithelial cells. Am J Physiol 276: L885-L889. (Pubitemid 29249642)
257. Wang R, Ibarra-Sunga O, Verlinski L, Pick R, Uhal BD, (2000a). Abrogation of bleomycin-induced epithelial apoptosis and lung fibrosis by captopril or by a caspase inhibitor. Am J Physiol Lung Cell Mol Physiol 279: L143-L151. (Pubitemid 30619534)
258. Wang WL, Healy ME, Sattler M, Verma S, Lin J, Maulik G, et al,. (2000b). Growth inhibition and modulation of kinase pathways of small cell lung cancer cell lines by the novel tyrosine kinase inhibitor STI 571. Oncogene 19: 3521-3528. (Pubitemid 30487449)
259. Watterson KR, Lanning DA, Diegelmann RF, Spiegel S, (2007). Regulation of fibroblast functions by lysophospholipid mediators: potential roles in wound healing. Wound Repair Regen 15: 607-616. (Pubitemid 47495881)
260. Whyte M, Hubbard R, Meliconi R, Whidborne M, Eaton V, Bingle C, et al,. (2000). Increased risk of fibrosing alveolitis associated with interleukin-1 receptor antagonist and tumor necrosis factor-alpha gene polymorphisms. Am J Respir Crit Care Med 162: 755-758. (Pubitemid 30647230)
261. Wilborn J, Crofford LJ, Burdick MD, Kunkel SL, Strieter RM, Peters-Golden M, (1995). Cultured lung fibroblasts isolated from patients with idiopathic pulmonary fibrosis have a diminished capacity to synthesize prostaglandin E2 and to express cyclooxygenase-2. J Clin Invest 95: 1861-1868.
262. Wilborn J, Bailie M, Coffey M, Burdick M, Strieter R, Peters-Golden M, (1996). Constitutive activation of 5-lipoxygenase in the lungs of patients with idiopathic pulmonary fibrosis. J Clin Invest 97: 1827-1836.
263. Willis BC, Liebler JM, Luby-Phelps K, Nicholson AG, Crandall ED, du Bois RM, et al,. (2005). Induction of epithelial-mesenchymal transition in alveolar epithelial cells by transforming growth factor-beta1: potential role in idiopathic pulmonary fibrosis. Am J Pathol 166: 1321-1332. (Pubitemid 40586504)
264. Wipff PJ, Hinz B, (2008). Integrins and the activation of latent transforming growth factor beta1-an intimate relationship. Eur J Cell Biol 87: 601-615.
265. Wipff PJ, Rifkin DB, Meister JJ, Hinz B, (2007). Myofibroblast contraction activates latent TGF-beta1 from the extracellular matrix. J Cell Biol 179: 1311-1323. (Pubitemid 350277747)
266. Wynn TA, (2004). Fibrotic disease and the T(H)1/T(H)2 paradigm. Nat Rev Immunol 4: 583-594.
267. Xaubet A, Marin-Arguedas A, Lario S, Ancochea J, Morell F, Ruiz-Manzano J, et al,. (2003). Transforming growth factor-beta1 gene polymorphisms are associated with disease progression in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 168: 431-435. (Pubitemid 37046871)
268. Xaubet A, Roca-Ferrer J, Pujols L, Ramirez J, Mullol J, Marin-Arguedas A, et al,. (2004). Cyclooxygenase-2 is up-regulated in lung parenchyma of chronic obstructive pulmonary disease and down-regulated in idiopathic pulmonary fibrosis. Sarcoidosis Vasc Diffuse Lung Dis 21: 35-42. (Pubitemid 38533812)
269. Xu S, Denton CP, Holmes A, Dashwood MR, Abraham DJ, Black CM, (1998). Endothelins: effect on matrix biosynthesis and proliferation in normal and scleroderma fibroblasts. J Cardiovasc Pharmacol 31 (Suppl. 1): S360-S363. (Pubitemid 128709951)
270. Xu YD, Hua J, Mui A, O'Connor R, Grotendorst G, Khalil N, (2003). Release of biologically active TGF-beta1 by alveolar epithelial cells results in pulmonary fibrosis. Am J Physiol Lung Cell Mol Physiol 285: L527-L539. (Pubitemid 37022852)
271. Xu MY, Porte J, Knox AJ, Weinreb PH, Maher TM, Violette SM, et al,. (2009). Lysophosphatidic acid induces alphavbeta6 integrin-mediated TGF-beta activation via the LPA2 receptor and the small G protein G alpha(q). Am J Pathol 174: 1264-1279.
272. Yamada M, Kuwano K, Maeyama T, Hamada N, Yoshimi M, Nakanishi Y, et al,. (2008). Dual-immunohistochemistry provides little evidence for epithelial-mesenchymal transition in pulmonary fibrosis. Histochem Cell Biol 129: 453-462.
273. Yanagisawa K, Osada H, Masuda A, Kondo M, Saito T, Yatabe Y, et al,. (1998). Induction of apoptosis by Smad3 and down-regulation of Smad3 expression in response to TGF-beta in human normal lung epithelial cells. Oncogene 17: 1743-1747. (Pubitemid 28482652)
274. Zappala CJ, Latsi PI, Nicholson AG, Colby TV, Cramer D, Renzoni EA, et al,. (2010). Marginal decline in forced vital capacity is associated with a poor outcome in idiopathic pulmonary fibrosis. Eur Respir J 35: 830-836.
275. Zavadil J, Cermak L, Soto-Nieves N, Bottinger EP, (2004). Integration of TGF-beta/Smad and Jagged1/Notch signalling in epithelial-to-mesenchymal transition. EMBO J 23: 1155-1165. (Pubitemid 38436879)
276. Zhang K, Gharaee-Kermani M, McGarry B, Phan SH, (1994a). In situ hybridization analysis of rat lung alpha 1(I) and alpha 2(I) collagen gene expression in pulmonary fibrosis induced by endotracheal bleomycin injection. Lab Invest 70: 192-202. (Pubitemid 24090101)
277. Zhang K, Rekhter MD, Gordon D, Phan SH, (1994b). Myofibroblasts and their role in lung collagen gene expression during pulmonary fibrosis. A combined immunohistochemical and in situ hybridization study. Am J Pathol 145: 114-125. (Pubitemid 24217490)
278. Zhang K, Gharaee-Kermani M, McGarry B, Remick D, Phan SH, (1997). TNF-alpha-mediated lung cytokine networking and eosinophil recruitment in pulmonary fibrosis. J Immunol 158: 954-959. (Pubitemid 127492676)
279. Zhang F, Nielsen LD, Lucas JJ, Mason RJ, (2004). Transforming growth factor-beta antagonizes alveolar type II cell proliferation induced by keratinocyte growth factor. Am J Respir Cell Mol Biol 31: 679-686. (Pubitemid 39587499)
280. Zhang Y, Liu P, Gao X, Qian W, Xu K, (2010). rAAV2-TGF-beta(3) decreases collagen synthesis and deposition in the liver of experimental hepatic fibrosis rat. Dig Dis Sci 55: 2821-2830.
281. Zhu Y, Liu Y, Zhou W, Xiang R, Jiang L, Huang K, et al,. (2010). A prostacyclin analogue, iloprost, protects from bleomycin-induced pulmonary fibrosis in mice. Respir Res 11: 34.
282. Ziegenhagen MW, Schrum S, Zissel G, Zipfel PF, Schlaak M, Muller-Quernheim J, (1998). Increased expression of proinflammatory chemokines in bronchoalveolar lavage cells of patients with progressing idiopathic pulmonary fibrosis and sarcoidosis. J Investig Med 46: 223-231. (Pubitemid 128452762)
283. Ziesche R, Hofbauer E, Wittmann K, Petkov V, Block LH, (1999). A preliminary study of long-term treatment with interferon gamma-1b and low-dose prednisolone in patients with idiopathic pulmonary fibrosis. N Engl J Med 341: 1264-1269. (Pubitemid 29489741)
284. Zisman DA, Lynch JP 3rd, Toews GB, Kazerooni EA, Flint A, Martinez FJ, (2000). Cyclophosphamide in the treatment of idiopathic pulmonary fibrosis: a prospective study in patients who failed to respond to corticosteroids. Chest 117: 1619-1626. (Pubitemid 30413354)
285. Zisman DA, Schwarz M, Anstrom KJ, Collard HR, Flaherty KR, Hunninghake GW, (2010). A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med 363: 620-628.
286. Zuo F, Kaminski N, Eugui E, Allard J, Yakhini Z, Ben-Dor A, et al,. (2002). Gene expression analysis reveals matrilysin as a key regulator of pulmonary fibrosis in mice and humans. Proc Natl Acad Sci USA 99: 6292-6297. (Pubitemid 34493305)