Letter to the editor 

**Guenther A, Eickelberg O, Preissner KT, Chambers R, Laurent G, Wells A, Crestani B, Vancheri B, Bonniaud P, Camus P, Schmitz G, Klepetko W, Schultze J, Vossmeyer D, Stumpf P. European IPF Network: Natural Course, Pathomechanisms and Novel Treatment Options in nolvadexIdiopathic Pulmonary Fibrosis.
Thorax 36:9, September 2008.  


**A. Guenther on behalf of the European IPF Network, Eur Respir J 37:1-3, 2011.

Letter to the editor

Loeh B, Drakopanagiotakis F, Bandelli GP, von der Beck D, Tello S, Cordani E, Rizza E, Barrocu L, Markart P, Seeger W, Günther A*, Albera C*. Intraindividual response to treatment with pirfenidone in Idiopathic Pulmonary Fibrosis (IPF). Am J Respir Crit Care Med 191(1):110-3; 2014


Amara N, Goven D, Prost F, Muloway R, Crestani B, Boczkowski J. NOX4/NADPH oxidase expression is increased in pulmonary fibroblasts from patients with idiopathic pulmonary fibrosis and only nowmediates TGFbeta1-induced fibroblast differentiation into myofibroblasts.  Thorax. 2010 Aug;65(8):733-8

Avcuoglu S, Wygrecka M, Marsh LM, Günther A, Seeger W, Weissmann N, Fink L, Morty RE, Kwapiszewska G. Neurotrophic tyrosine kinase receptor B/neurotrophin 4 signaling axis is perturbed in clinical and experimental pulmonary fibrosis. Am J Respir Cell Mol Biol. 2011 Oct;45(4):768-80

Bantsimba-Malanda C, Marchal-Sommé J, Goven D, Freynet O, Michel L, Crestani B, Soler P. A role for dendritic cells in bleomycin-induced pulmonary fibrosis in mice? Am J Respir Crit Care Med. 2010 Aug 1;182(3):385-95

Cigna N, Farrokhi Moshai E, Brayer S, Marchal-Somme J, Wémeau-Stervinou L, Fabre A, Mal H, Lesèche G, Dehoux M, Soler P, Crestani B, Mailleux AA. The hedgehog system machinery controls transforming growth factor-β-dependent myofibroblastic differentiation in humans: involvement in idiopathic pulmonary fibrosis. Am J Pathol. 2012 Dec;181(6):2126-37

**Conte E, Gili E, Fruciano M, Korfei M, Fagone E, Iemmolo M, Lo Furno D, Giuffrida R, Crimi N, Guenther A, Vancheri C. PI3K p110γ overexpression in idiopathic pulmonary fibrosis lung tissue and fibroblast cells: in vitro effects of its inhibition. Lab Invest. 2013 May;93(5):566-76

Conte E, Fruciano M, Fagone E, Gili E, Caraci F, Iemmolo M, Crimi N, Vancheri C. Inhibition of PI3K Prevents the Proliferation and Differentiation of Human Lung Fibroblasts into Myofibroblasts: The Role of Class I P110 Isoforms. PLoS One. 2011;6(10):e24663

Datta A, Scotton CJ, Chambers RC. Novel therapeutic approaches for pulmonary fibrosis. Br J Pharmacol. 2011 May;163(1):141-72

Decologne N, Wettstein G, Kolb M, Margetts P, Garrido C, Camus P, Bonniaud P. Bleomycin induces pleural and subpleural fibrosis in the presence of carbon particles. Eur Respir J. 2010 Jan;35(1):176-85

Decologne N, Wettstein G, Bonniaud P. A role for mesothelial cells in the genesis of idiopathic pulmonary fibrosis? Bull Acad Natl Med. 2010 Feb;194(2):383-9.

Ecker J, Liebisch G, Englmaier M, Grandl, Schmitz G. SREBP-1c mediated induction of lipid metabolism is crucial for phagocytic differentiation of monocytes. Chemistry of Physics of Lipids. 2009 160:S20

Failla M, Genovese T, Mazzon E, Fruciano M, Fagone E, Gili E, Barera A, La Rosa C, Conte E, Crimi N, Cuzzocrea S, Vancheri C. 16,16-Dimethyl prostaglandin E2 efficacy on prevention and protection from bleomycin-induced lung injury and fibrosis. Am J Respir Cell Mol Biol. 2009 Jul;41(1):50-8.

Fagone E, Conte E, Gili E, Fruciano M, Pistorio MP, Lo Furno D, Giuffrida R, Crimi N, Vancheri C. Resveratrol inhibits transforming growth factor-β–induced proliferation and differentiation of ex vivo human lung fibroblasts into myofibroblasts through ERK/Akt inhibition and PTEN restoration. Exp Lung Res. 2011 Apr;37(3):162-74.

Gupte VV, Ramasamy SK, Reddy R, Lee J, Weinreb PH, Violette SM, Guenther A, Warburton D, Driscoll B, Minoo P, Bellusci S. Overexpression of fibroblast growth factor-10 during both inflammatory and fibrotic phases attenuates bleomycin-induced pulmonary fibrosis in mice.

Günther A, Korfei M, Mahavadi P, von der Beck D, Ruppert C, Markart P. Unravelling the progressive pathophysiology of idiopathic pulmonary fibrosis. Eur Respir Rev. 2012 Jun 1;21(124):152-60. Am J Respir Crit Care Med. 2009 Sep 1;180(5):424-36

Janssen W, Pullamsetti SS, Cooke J, Weissmann N, Guenther A, Schermuly RT. The role of dimethylarginine dimethylaminohydorlase (DDAH) in pulmonary fibrosis. J Pathol 2013 229(2):242-249

Königshoff M, Kramer M, Balsara N, Wilhelm J, Amarie OV, Jahn A, Rose F, Fink L, Seeger W, Schaefer L, Günther A, Eickelberg O. WNT1-inducible signaling protein-1 mediates pulmonary fibrosis in mice and is upregulated in humans with idiopathic pulmonary fibrosis. J Clin Invest. 2009 Apr;119(4):772-87

**Korfei M, von der Beck D, Henneke I, Markart P, Ruppert C, Mahavadi P, Ghanim B, Klepetko W, Fink L, Meiners S, Krämer OH, Seeger W, Vancheri C, Guenther A. Comparative proteome analysis of lung tissue from patients with idiopathic pulmonary fibrosis (IPF), non-specific interstitial pneumonia (NSIP) and organ donors. J Proteomics. 2013 Jun 24;85:109-28

**Korfei M, Schmitt S, Ruppert C, Henneke I, Markart P, Loeh B, Mahavadi P, Wygrecka M, Klepetko W, Fink L, Bonniaud P, Preissner KT, Lochnit G, Schaefer L, Seeger W, Guenther A. Comparative proteomic analysis of lung tissue from patients with idiopathic pulmonary fibrosis (IPF) and lung transplant donor lungs. J Proteome Res. 2011 May 6;10(5):2185-205

**Mahavadi P, Korfei M, Henneke I, Liebisch G, Schmitz G, Gochuico BR, Markart P, Bellusci S, Seeger W, Ruppert C, Guenther A. Epithelial Stress and Apoptosis Underlie Hermansky-Pudlak Syndrome–associated Interstitial Pneumonia. Am J Respir Crit Care Med. 2010 Jul 15;182(2):207-19

Markart P, Nass R, Ruppert C, Hundack L, Wygrecka M, Korfei M, Boedeker RH, Staehler G, Kroll H, Scheuch G, Seeger W, Guenther A. Safety and tolerability of inhaled heparin in idiopathic pulmonary fibrosis. J Aerosol Med Pulm Drug Deliv. 2010 Jun;23(3):161-72

**Nikolova S, Guenther A, Savai R, Weissmann N, Ghofrani HA, Konigshoff M, Eickelberg O, Klepetko W, Voswinckel R, Seeger W, Grimminger F, Schermuly RT, Pullamsetti SS. Phosphodiesterase 6 subunits are expressed and altered in idiopathic pulmonary fibrosis. Respir Res. 2010 Oct 27;11:146

**Nkyimbeng T, Ruppert C, Shiomi T, Dahal B, Lang G, Seeger W, Okada Y, DÁrmiento J, Günther A. Pivotal role of Matrix Mtealloproteinase 13 in extracellular matrix turnover in Idiopathic Pulmonary Fibrosis. PLoS One. Accepted for publication July 2013

Ortiz-Stern A, Deng X, Smoktunowicz N, Mercer PF, Chambers RC. PAR-1-dependent and PAR-independent pro-inflammatory signalling in human lung fibroblasts exposed to thrombin. J Cell Physiol. 2012 Nov;227(11):3575-84

**Plantier L, Crestani B, Wert SE, Dehoux M, Zweytick B, Guenther A, Whitsett JA. Ectopic respiratory epithelial cell differentiation in bronchiolised distal airspaces in idiopathic pulmonary fibrosis. Thorax. 2011 Aug;66(8):651-7

Pfaff EM, Becker S, Günther A, Königshoff M. Dickkopf proteins influence lung epithelial cell proliferation in idiopathic pulmonary fibrosis. Eur Respir J. 2011 Jan;37(1):79-87

Phin S, Marchand-Adam S, Fabre A, Marchal-Somme J, Bantsimba-Malanda C, Kataoka H, Soler P, Crestani B. Imbalance in the pro-hepatocyte growth factor activation system in bleomycin-induced lung fibrosis in mice. Am J Respir Cell Mol Biol. 2010 Mar;42(3):286-93

**Pullamsetti SS, Savai R, Dumitrascu R, Dahal BK, Wilhelm J, Konigshoff M, Zakrzewicz D, Ghofrani HA, Weissmann N, Eickelberg O, Guenther A, Leiper J, Seeger W, Grimminger F, Schermuly RT. The role of dimethylarginine dimethylaminohydrolase in idiopathic pulmonary fibrosis. Sci Transl Med. 2011 Jun 15;3(87):87ra53

Quesnel C, Piednoir P, Gelly J, Nardelli L, Garnier M, Leçon V, Lasocki S, Bouadma L, Philip I, Elbim C, Mentré F, Soler P, Crestani B, Dehoux M. Alveolar fibrocyte percentage is an independent predictor of poor outcome in patients with acute lung injury. Crit Care Med. 2012 Jan;40(1):21-8

Quesnel C, Nardelli L, Piednoir P, Leçon V, Marchal-Somme J, Lasocki S, Bouadma L, Philip I, Soler P, Crestani B, Dehoux M. Alveolar fibroblasts in acute lung injury: biological behaviour and clinical relevance. Eur Respir J. 2010 Jun;35(6):1312-21

Ruppert C, Kuchenbuch T, Boensch M, Schmidt S, Mathes U, Hillebrand V, Henneke I, Markart P, Reiss I, Schermuly RT, Seeger W, Günther A. Dry powder aerosolization of a recombinant surfactant protein-C-based surfactant for inhalative treatment of the acutely inflamed lung. Crit Care Med. 2010 Jul;38(7):1584-91

Scherer M, Leuthäuser-Jaschinski K, Ecker J, Schmitz G, Liebisch G. A rapid and quantitative LC-MS/MS method to profile sphingolipids. J Lipid Res. 2010 Jul;51(7):2001-11

**Scotton CJ, Krupiczojc MA, Königshoff M, Mercer PF, Lee YC, Kaminski N, Morser J, Post JM, Maher TM, Nicholson AG, Moffatt JD, Laurent GJ, Derian CK, Eickelberg O, Chambers RC. Increased local expression of coagulation factor X contributes to the fibrotic response in human and murine lung injury. J Clin Invest. 2009 Sep;119(9):2550-63

Scotton CJ, Chambers RC. Bleomycin revisited: towards a more representative model of IPF? Am J Physiol Lung Cell Mol Physiol. 2010 Oct;299(4):L439-41. Comment on Repetitive intratracheal bleomycin models several features of idiopathic pulmonary fibrosis.

Shetty S, Bhandary YP, Shetty SK, Velusamy T, Shetty P, Bdeir K, Gyetko MR, Cines DB, Idell S, Neuenschwander PF, Ruppert C, Guenther A, Abraham E, Shetty RS. Induction of tissue factor by urokinase in lung epithelial cells and in the lungs. Am J Respir Crit Care Med. 2010 Jun 15;181(12):1355-66

Taillé C, Grootenboer-Mignot S, Boursier C, Michel L, Debray MP, Fagart J, Barrientos L, Mailleux A, Cigna N, Tubach F, Marchal-Sommé J, Soler P, Chollet-Martin S, Crestani B. Identification of periplakin as a new target for autoreactivity in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011 Mar 15;183(6):759-66

Udalov S, Dumitrascu R, Pullamsetti SS, Al-tamari HM, Weissmann N, Ghofrani HA, Guenther A, Voswinckel R, Seeger W, Grimminger F, Schermuly RT. Effects of phosphodiesterase 4 inhibition on bleomycin-induced pulmonary fibrosis in mice. BMC Pulm Med. 2010 May 5;10:26

**Wettstein G, Bellaye PS, Kolb M, Hammann A, Crestani B, Soler P, Marchal-Somme J, Hazoume A, Gauldie J, Gunther A, Micheau O, Gleave M, Camus P, Garrido C, Bonniaud P. Inhibition of HSP27 blocks fibrosis development and EMT features by promoting Snail degradation. FASEB J. 2013 Apr;27(4):1549-60

Wettstein G, Bellaye PS, Micheau O, Bonniaud P. Small heat shock proteins and the cytoskeleton: an essential interplay for cell integrity? Int J Biochem Cell Biol. 2012 Oct;44(10):1680-6

Wygrecka M, Kwapiszewska G, Jablonska E, von Gerlach S, Henneke I, Zakrzewicz D, Guenther A, Preissner KT, Markart P. Role of protease-activated receptor-2 in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011 Jun 15;183(12):1703-14



** indicates joint publication of consortium members