{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T14:27:40Z","timestamp":1788791260965,"version":"build-2803163510"},"reference-count":87,"publisher":"Georg Thieme Verlag KG","issue":"04","funder":[{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"crossref","award":["1R01 HL134801 ,1R01 HL157176"],"award-info":[{"award-number":["1R01 HL134801 ,1R01 HL157176"]}],"id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Semin Respir Crit Care Med"],"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Pulmonary fibrosis is characterized by scarring and thickening of the lung parenchyma due to excessive deposition of collagen and other extracellular matrix (ECM) proteins. This leads to disruption of gas exchange areas and ultimately respiratory failure, a pathology shared across multiple interstitial lung diseases (ILDs). Idiopathic pulmonary fibrosis (IPF) is a chronic and progressive ILD characterized by exertional dyspnea, dry cough, and restrictive lung defects. Clinical progression is marked by worsening lung function, declining exercise tolerance, and hypoxemia. High-resolution computed tomography in IPF typically shows reticular opacities and honeycombing, predominantly distributed in the subpleural regions and lower lobes of the lungs. The disease course is variable, with episodes of acute exacerbation associated with high mortality. Myofibroblasts and fibroblasts are central drivers of fibrogenesis through uncontrolled proliferation, migration, survival, senescence, myofibroblast differentiation, and ECM production. Myofibroblasts represent a heterogeneous population in both origin and function, arising from diverse precursor cells, including lung resident fibroblasts, endothelial cells, and mesothelial cells, and are shaped by tissue-specific niches. Persistent activation of (myo)fibroblasts is sustained by a complex network of profibrotic growth factors and their downstream transcriptional regulators. In this review, we comprehensively examine the cellular origins and molecular pathways underlying fibroblast activation, with an emphasis on mechanistic insights that may inform the development of targeted antifibrotic therapies to attenuate disease progression and improve patient outcomes.<\/jats:p>","DOI":"10.1055\/a-2649-9359","type":"journal-article","created":{"date-parts":[[2025,7,24]],"date-time":"2025-07-24T23:40:42Z","timestamp":1753400442000},"page":"322-329","source":"Crossref","is-referenced-by-count":4,"title":["Emerging Concepts in Fibroblast Biology and Progressive Pulmonary Fibrosis"],"prefix":"10.1055","volume":"46","author":[{"given":"Priyanka","family":"Singh","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shanda","family":"Edjah","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wei","family":"Shi","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satish K.","family":"Madala","sequence":"additional","affiliation":[{"name":"Division of Pulmonary, Critical Care and Sleep Medicine, Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2025,8,12]]},"reference":[{"issue":"06","key":"ref1","doi-asserted-by":"crossref","first-page":"1721","DOI":"10.1183\/13993003.02115-2015","article-title":"Prevalence and characteristics of TERT and TERC mutations in suspected genetic pulmonary fibrosis","volume":"48","author":"R Borie","year":"2016","journal-title":"Eur Respir J"},{"issue":"08","key":"ref2","doi-asserted-by":"crossref","first-page":"1232","DOI":"10.3390\/jcm8081232","article-title":"Telomere abnormalities in the pathobiology of idiopathic pulmonary fibrosis","volume":"8","author":"H Bilgili","year":"2019","journal-title":"J Clin Med"},{"issue":"02","key":"ref3","doi-asserted-by":"crossref","first-page":"460","DOI":"10.1378\/chest.14-0867","article-title":"The MUC5B promoter polymorphism is associated with idiopathic pulmonary fibrosis in a Mexican cohort but is rare among Asian ancestries","volume":"147","author":"A L Peljto","year":"2015","journal-title":"Chest"},{"issue":"01","key":"ref4","doi-asserted-by":"crossref","first-page":"2102147","DOI":"10.1183\/13993003.02147-2021","article-title":"Surfactant protein C mutations and familial pulmonary fibrosis: stuck in a loop on the scenic route","volume":"59","author":"B Gooptu","year":"2022","journal-title":"Eur Respir J"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"1013526","DOI":"10.3389\/fcimb.2022.1013526","article-title":"Pulmonary fibrosis in patients with COVID-19: A retrospective study","volume":"12","author":"F Li","year":"2022","journal-title":"Front Cell Infect Microbiol"},{"issue":"03","key":"ref6","doi-asserted-by":"crossref","first-page":"1047","DOI":"10.1152\/physrev.00048.2006","article-title":"Pathobiology of cigarette smoke-induced chronic obstructive pulmonary disease","volume":"87","author":"T Yoshida","year":"2007","journal-title":"Physiol Rev"},{"issue":"01","key":"ref7","doi-asserted-by":"crossref","first-page":"496","DOI":"10.1186\/s12967-021-03159-9","article-title":"Virus infection induced pulmonary fibrosis","volume":"19","author":"W J Huang","year":"2021","journal-title":"J Transl Med"},{"issue":"08","key":"ref8","doi-asserted-by":"crossref","first-page":"2028","DOI":"10.3390\/diagnostics12082028","article-title":"Interstitial lung fibrosis following COVID-19 pneumonia","volume":"12","author":"M Lazar","year":"2022","journal-title":"Diagnostics (Basel)"},{"issue":"06","key":"ref9","doi-asserted-by":"crossref","first-page":"650","DOI":"10.1164\/rccm.201903-0563CI","article-title":"Etiology, risk factors, and biomarkers in systemic sclerosis with interstitial lung disease","volume":"201","author":"D Khanna","year":"2020","journal-title":"Am J Respir Crit Care Med"},{"issue":"01","key":"ref10","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1111\/bjd.17886","article-title":"Anti-C1q autoantibodies are frequently detected in patients with systemic sclerosis associated with pulmonary fibrosis","volume":"181","author":"C Liaskos","year":"2019","journal-title":"Br J Dermatol"},{"issue":"163","key":"ref11","doi-asserted-by":"crossref","first-page":"210106","DOI":"10.1183\/16000617.0106-2021","article-title":"Extracellular vesicle-mediated cellular crosstalk in lung repair, remodelling and regeneration","volume":"31","author":"T Kadota","year":"2022","journal-title":"Eur Respir Rev"},{"issue":"03","key":"ref12","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1111\/imm.13319","article-title":"Stromal-immune cell crosstalk fundamentally alters the lung microenvironment following tissue insult","volume":"163","author":"J C Worrell","year":"2021","journal-title":"Immunology"},{"issue":"07","key":"ref13","doi-asserted-by":"crossref","first-page":"1339","DOI":"10.1084\/jem.20110551","article-title":"Integrating mechanisms of pulmonary fibrosis","volume":"208","author":"T A Wynn","year":"2011","journal-title":"J Exp Med"},{"issue":"16","key":"ref14","doi-asserted-by":"crossref","first-page":"e121252","DOI":"10.1172\/jci.insight.121252","article-title":"Wilms' tumor 1 drives fibroproliferation and myofibroblast transformation in severe fibrotic lung disease","volume":"3","author":"V Sontake","year":"2018","journal-title":"JCI Insight"},{"issue":"01","key":"ref15","doi-asserted-by":"crossref","first-page":"00571","DOI":"10.1183\/23120541.00571-2021","article-title":"Vascular remodelling in idiopathic pulmonary fibrosis patients and its detrimental effect on lung physiology: potential role of endothelial-to-mesenchymal transition","volume":"8","author":"A V Gaikwad","year":"2022","journal-title":"ERJ Open Res"},{"issue":"05","key":"ref16","doi-asserted-by":"crossref","first-page":"2660","DOI":"10.1016\/j.ajpath.2011.07.042","article-title":"Inflammation-induced endothelial-to-mesenchymal transition: a novel mechanism of intestinal 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Valenzi","year":"2019","journal-title":"Ann Rheum Dis"},{"issue":"02","key":"ref20","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1016\/j.ydbio.2011.06.011","article-title":"Wnt2 signaling is necessary and sufficient to activate the airway smooth muscle program in the lung by regulating myocardin\/Mrtf-B and Fgf10 expression","volume":"356","author":"A M Goss","year":"2011","journal-title":"Dev Biol"},{"issue":"7464","key":"ref21","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1038\/nature12358","article-title":"Coordination of heart and lung co-development by a multipotent cardiopulmonary progenitor","volume":"500","author":"T Peng","year":"2013","journal-title":"Nature"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1186\/1741-7007-11-111","article-title":"Spatial-temporal targeting of lung-specific mesenchyme by a Tbx4 enhancer","volume":"11","author":"W Zhang","year":"2013","journal-title":"BMC 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Defining a mesenchymal progenitor niche at single-cell resolution","volume":"346","author":"M E Kumar","year":"2014","journal-title":"Science"},{"issue":"08","key":"ref25","doi-asserted-by":"crossref","first-page":"3063","DOI":"10.1172\/JCI85328","article-title":"Transcription factor TBX4 regulates myofibroblast accumulation and lung fibrosis","volume":"126","author":"T Xie","year":"2016","journal-title":"J Clin Invest"},{"issue":"05","key":"ref26","doi-asserted-by":"crossref","first-page":"842","DOI":"10.1002\/ajmg.a.61096","article-title":"Identification of a deletion containing TBX4 in a neonate with acinar dysplasia by rapid exome sequencing","volume":"179","author":"K German","year":"2019","journal-title":"Am J Med Genet A"},{"issue":"08","key":"ref27","doi-asserted-by":"crossref","first-page":"e1002866","DOI":"10.1371\/journal.pgen.1002866","article-title":"Multiple roles and interactions of Tbx4 and Tbx5 in development of the respiratory system","volume":"8","author":"R Arora","year":"2012","journal-title":"PLoS Genet"},{"issue":"06","key":"ref28","doi-asserted-by":"crossref","first-page":"1239","DOI":"10.1086\/421331","article-title":"Mutations in the human TBX4 gene cause small patella syndrome","volume":"74","author":"E M Bongers","year":"2004","journal-title":"Am J Hum Genet"},{"issue":"03","key":"ref29","doi-asserted-by":"crossref","first-page":"384","DOI":"10.1002\/path.4530","article-title":"A novel profibrotic mechanism mediated by TGF\u03b2-stimulated collagen prolyl hydroxylase expression in fibrotic lung mesenchymal cells","volume":"236","author":"Y Luo","year":"2015","journal-title":"J Pathol"},{"issue":"09","key":"ref30","doi-asserted-by":"crossref","first-page":"L942","DOI":"10.1152\/ajplung.00272.2014","article-title":"Characterization of the platelet-derived growth factor receptor-\u03b1-positive cell lineage during murine late lung development","volume":"309","author":"A Ntokou","year":"2015","journal-title":"Am J Physiol Lung Cell 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Ruiz-Villalba","year":"2020","journal-title":"Circulation"},{"issue":"03","key":"ref40","doi-asserted-by":"crossref","first-page":"102934","DOI":"10.1016\/j.jbc.2023.102934","article-title":"The well-developed actin cytoskeleton and Cthrc1 expression by actin-binding protein drebrin in myofibroblasts promote cardiac and hepatic fibrosis","volume":"299","author":"T Hironaka","year":"2023","journal-title":"J Biol Chem"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"43","DOI":"10.1016\/j.ebiom.2019.01.009","article-title":"Autocrine CTHRC1 activates hepatic stellate cells and promotes liver fibrosis by activating TGF-\u03b2 signaling","volume":"40","author":"J Li","year":"2019","journal-title":"EBioMedicine"},{"issue":"06","key":"ref42","first-page":"814","article-title":"Cancer-associated fibroblast-secreted collagen triple helix repeat containing-1 promotes breast cancer cell migration, invasiveness and epithelial-mesenchymal transition by activating the Wnt\/\u03b2-catenin pathway","volume":"22","author":"H Li","year":"2021","journal-title":"Oncol Lett"},{"issue":"05","key":"ref43","doi-asserted-by":"crossref","first-page":"e13115","DOI":"10.14814\/phy2.13115","article-title":"Cthrc1 lowers pulmonary collagen associated with bleomycin-induced fibrosis and protects lung function","volume":"5","author":"A P Binks","year":"2017","journal-title":"Physiol Rep"},{"issue":"07","key":"ref44","doi-asserted-by":"crossref","first-page":"588","DOI":"10.1164\/rccm.200810-1534OC","article-title":"Circulating fibrocytes are an indicator of poor prognosis in idiopathic pulmonary fibrosis","volume":"179","author":"A Moeller","year":"2009","journal-title":"Am J Respir Crit Care Med"},{"issue":"01","key":"ref45","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1186\/s12931-018-0798-8","article-title":"Fibrocytes are increased in lung and peripheral blood of patients with idiopathic pulmonary fibrosis","volume":"19","author":"P Heukels","year":"2018","journal-title":"Respir Res"},{"issue":"01","key":"ref46","doi-asserted-by":"crossref","first-page":"e53736","DOI":"10.1371\/journal.pone.0053736","article-title":"Detection of alveolar fibrocytes in idiopathic pulmonary fibrosis and systemic sclerosis","volume":"8","author":"R Borie","year":"2013","journal-title":"PLoS One"},{"issue":"04","key":"ref47","doi-asserted-by":"crossref","first-page":"777","DOI":"10.1165\/rcmb.2013-0042OC","article-title":"Bone marrow-derived stromal cells are invasive and hyperproliferative and alter transforming growth factor-\u03b1-induced pulmonary fibrosis","volume":"50","author":"S K Madala","year":"2014","journal-title":"Am J Respir Cell Mol Biol"},{"key":"ref48","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1007\/978-3-030-35723-8_6","article-title":"Fibrocytes in the tumor microenvironment","volume":"1224","author":"D Roife","year":"2020","journal-title":"Adv Exp Med Biol"},{"issue":"02","key":"ref49","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1586\/17476348.2014.862154","article-title":"Paracrine functions of fibrocytes to promote lung fibrosis","volume":"8","author":"K R Kleaveland","year":"2014","journal-title":"Expert Rev Respir Med"},{"issue":"38","key":"ref50","doi-asserted-by":"crossref","first-page":"11929","DOI":"10.1073\/pnas.1507387112","article-title":"TNF-\u03b1-stimulated fibroblasts secrete lumican to promote fibrocyte differentiation","volume":"112","author":"D Pilling","year":"2015","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"08","key":"ref51","doi-asserted-by":"crossref","first-page":"3978","DOI":"10.4049\/jimmunol.1500963","article-title":"Fibrocytes regulate Wilms tumor 1-positive cell accumulation in severe fibrotic lung disease","volume":"195","author":"V Sontake","year":"2015","journal-title":"J Immunol"},{"issue":"04","key":"ref53","doi-asserted-by":"crossref","first-page":"L607","DOI":"10.1152\/ajplung.00392.2021","article-title":"Effect of lung pericyte-like cell ablation on the bleomycin model of injury and repair","volume":"322","author":"C F Hung","year":"2022","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"issue":"07","key":"ref54","doi-asserted-by":"crossref","first-page":"820","DOI":"10.1164\/rccm.201212-2297OC","article-title":"Role of lung pericytes and resident fibroblasts in the pathogenesis of pulmonary fibrosis","volume":"188","author":"C Hung","year":"2013","journal-title":"Am J Respir Crit Care Med"},{"issue":"01","key":"ref55","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1183\/09031936.00141010","article-title":"Parenchymal trafficking of pleural mesothelial cells in idiopathic pulmonary fibrosis","volume":"39","author":"K K Mubarak","year":"2012","journal-title":"Eur Respir J"},{"issue":"02","key":"ref56","doi-asserted-by":"crossref","first-page":"244","DOI":"10.1165\/rcmb.2014-0011OC","article-title":"Antifibrotic role of \u03b1B-crystallin inhibition in pleural and subpleural fibrosis","volume":"52","author":"P S Bellaye","year":"2015","journal-title":"Am J Respir Cell Mol Biol"},{"issue":"09","key":"ref57","doi-asserted-by":"crossref","first-page":"1455","DOI":"10.1089\/scd.2011.0219","article-title":"Endothelial differentiation by multipotent fetal mouse lung mesenchymal cells","volume":"21","author":"Y Yamamoto","year":"2012","journal-title":"Stem Cells Dev"},{"issue":"02","key":"ref58","doi-asserted-by":"crossref","first-page":"00487","DOI":"10.1183\/23120541.00487-2022","article-title":"Endothelial-to-mesenchymal transition: a precursor to pulmonary arterial remodelling in patients with idiopathic pulmonary fibrosis","volume":"9","author":"A V Gaikwad","year":"2023","journal-title":"ERJ Open Res"},{"issue":"01","key":"ref59","doi-asserted-by":"crossref","first-page":"247","DOI":"10.4049\/jimmunol.1800883","article-title":"Macrophages guard endothelial lineage by hindering endothelial-to-mesenchymal transition: implications for the pathogenesis of systemic sclerosis","volume":"203","author":"P A Nicolosi","year":"2019","journal-title":"J Immunol"},{"issue":"01","key":"ref60","doi-asserted-by":"crossref","first-page":"5449","DOI":"10.1038\/s41467-024-49545-x","article-title":"Lung injury-induced activated endothelial cell states persist in aging-associated progressive fibrosis","volume":"15","author":"A A Raslan","year":"2024","journal-title":"Nat Commun"},{"key":"ref61","first-page":"724080","article-title":"Roles of TGF-\u03b2 signals in endothelial-mesenchymal transition during cardiac fibrosis","volume":"2011","author":"Y Yoshimatsu","year":"2011","journal-title":"Int J Inflamm"},{"issue":"35","key":"ref62","doi-asserted-by":"crossref","first-page":"13180","DOI":"10.1073\/pnas.0605669103","article-title":"Alveolar epithelial cell mesenchymal transition develops in vivo during pulmonary fibrosis and is regulated by the extracellular matrix","volume":"103","author":"K K Kim","year":"2006","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"02","key":"ref63","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1164\/rccm.201002-0279OC","article-title":"An essential role for resident fibroblasts in experimental lung fibrosis is defined by lineage-specific deletion of high-affinity type II transforming growth factor \u03b2 receptor","volume":"183","author":"R K Hoyles","year":"2011","journal-title":"Am J Respir Crit Care Med"},{"issue":"01","key":"ref64","doi-asserted-by":"crossref","first-page":"4254","DOI":"10.1038\/s41467-020-18047-x","article-title":"Fibroblast-enriched endoplasmic reticulum protein TXNDC5 promotes pulmonary fibrosis by augmenting TGF\u03b2 signaling through TGFBR1 stabilization","volume":"11","author":"T-H Lee","year":"2020","journal-title":"Nat Commun"},{"issue":"01","key":"ref65","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1186\/s12931-020-1319-0","article-title":"TGF-\u03b2 pathway activation by idiopathic pulmonary fibrosis (IPF) fibroblast derived soluble factors is mediated by IL-6 trans-signaling","volume":"21","author":"G Epstein Shochet","year":"2020","journal-title":"Respir Res"},{"issue":"03","key":"ref66","doi-asserted-by":"crossref","first-page":"130","DOI":"10.1513\/pats.201201-003AW","article-title":"TGF-\u03b2 activation and lung fibrosis","volume":"9","author":"A L Tatler","year":"2012","journal-title":"Proc Am Thorac Soc"},{"issue":"52","key":"ref67","doi-asserted-by":"crossref","first-page":"43000","DOI":"10.1074\/jbc.M510441200","article-title":"Proliferation of pulmonary interstitial fibroblasts is mediated by transforming growth factor-\u03b21-induced release of extracellular fibroblast growth factor-2 and phosphorylation of p38 MAPK and JNK","volume":"280","author":"N Khalil","year":"2005","journal-title":"J Biol Chem"},{"issue":"03","key":"ref68","doi-asserted-by":"crossref","first-page":"L585","DOI":"10.1152\/ajplung.00151.2001","article-title":"Smad3 deficiency attenuates bleomycin-induced pulmonary fibrosis in mice","volume":"282","author":"J Zhao","year":"2002","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"issue":"S 01","key":"ref69","doi-asserted-by":"crossref","first-page":"S21","DOI":"10.1513\/AnnalsATS.201406-245MG","article-title":"Epithelial-mesenchymal interactions in fibrosis and repair. Transforming growth factor-\u03b2 activation by epithelial cells and fibroblasts","volume":"12","author":"D Sheppard","year":"2015","journal-title":"Ann Am Thorac Soc"},{"issue":"05","key":"ref70","doi-asserted-by":"crossref","first-page":"562","DOI":"10.1165\/rcmb.2008-0377OC","article-title":"Rapamycin prevents transforming growth factor-\u03b1-induced pulmonary fibrosis","volume":"41","author":"T R Korfhagen","year":"2009","journal-title":"Am J Respir Cell Mol Biol"},{"issue":"03","key":"ref71","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1667\/RR13625.1","article-title":"Transforming growth factor alpha is a critical mediator of radiation lung injury","volume":"182","author":"E J Chung","year":"2014","journal-title":"Radiat Res"},{"issue":"14","key":"ref72","doi-asserted-by":"crossref","first-page":"2769","DOI":"10.4161\/cc.9.14.12268","article-title":"Signaling pathways in the epithelial origins of pulmonary fibrosis","volume":"9","author":"W D Hardie","year":"2010","journal-title":"Cell Cycle"},{"issue":"06","key":"ref73","doi-asserted-by":"crossref","first-page":"L1217","DOI":"10.1152\/ajplung.00020.2008","article-title":"EGF receptor tyrosine kinase inhibitors diminish transforming growth factor-\u03b1-induced pulmonary fibrosis","volume":"294","author":"W D Hardie","year":"2008","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"issue":"01","key":"ref74","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1186\/s12931-014-0157-3","article-title":"Anti-fibrotic effects of nintedanib in lung fibroblasts derived from patients with idiopathic pulmonary fibrosis","volume":"15","author":"K E Hostettler","year":"2014","journal-title":"Respir Res"},{"issue":"12","key":"ref75","doi-asserted-by":"crossref","first-page":"e0209786","DOI":"10.1371\/journal.pone.0209786","article-title":"Blockade of platelet-derived growth factor receptor-\u03b2, not receptor-\u03b1 ameliorates bleomycin-induced pulmonary fibrosis in mice","volume":"13","author":"M Kishi","year":"2018","journal-title":"PLoS One"},{"issue":"06","key":"ref76","doi-asserted-by":"crossref","first-page":"925","DOI":"10.1084\/jem.20041393","article-title":"Inhibition of platelet-derived growth factor signaling attenuates pulmonary fibrosis","volume":"201","author":"A Abdollahi","year":"2005","journal-title":"J Exp Med"},{"issue":"06","key":"ref77","doi-asserted-by":"crossref","first-page":"1220","DOI":"10.1183\/09031936.01.00074101","article-title":"Type II alveolar epithelial cells and interstitial fibroblasts express connective tissue growth factor in IPF","volume":"17","author":"L H Pan","year":"2001","journal-title":"Eur Respir J"},{"issue":"01","key":"ref78","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1186\/s12931-015-0242-2","article-title":"Increased FGF1-FGFRc expression in idiopathic pulmonary fibrosis","volume":"16","author":"B MacKenzie","year":"2015","journal-title":"Respir 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