{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T19:53:12Z","timestamp":1790020392298,"version":"4.0.1"},"reference-count":43,"publisher":"Public Library of Science (PLoS)","issue":"8","license":[{"start":{"date-parts":[[2013,8,12]],"date-time":"2013-08-12T00:00:00Z","timestamp":1376265600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0070196","type":"journal-article","created":{"date-parts":[[2013,8,12]],"date-time":"2013-08-12T21:20:09Z","timestamp":1376342409000},"page":"e70196","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":64,"title":["Myofibroblast Differentiation and Enhanced Tgf-B Signaling in Cystic Fibrosis Lung Disease"],"prefix":"10.1371","volume":"8","author":[{"given":"William T.","family":"Harris","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David R.","family":"Kelly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yong","family":"Zhou","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dezhi","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark","family":"Macewen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James S.","family":"Hagood","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J. P.","family":"Clancy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Namasivayam","family":"Ambalavanan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric J.","family":"Sorscher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2013,8,12]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"918","DOI":"10.1164\/rccm.200304-505SO","article-title":"Pathophysiology and management of pulmonary infections in cystic fibrosis","volume":"168","author":"RL Gibson","year":"2003","journal-title":"Am J Respir Crit Care Med"},{"key":"ref2","unstructured":"CFF Registry Data (2008)"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"416","DOI":"10.1097\/01.mcp.0000245707.59138.40","article-title":"Gene modifiers of lung disease","volume":"12","author":"MR Knowles","year":"2006","journal-title":"Curr Opin Pulm Med"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"20571","DOI":"10.1073\/pnas.1015281107","article-title":"Pigs and humans with cystic fibrosis have reduced insulin-like growth factor 1 (IGF1) levels at birth","volume":"107","author":"MP Rogan","year":"2010","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"29ra31","DOI":"10.1126\/scitranslmed.3000928","article-title":"Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth","volume":"2","author":"DA Stoltz","year":"2010","journal-title":"Sci Transl Med"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"1251","DOI":"10.1164\/rccm.201004-0643OC","article-title":"Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children","volume":"182","author":"DK Meyerholz","year":"2010","journal-title":"Am J Respir Crit Care Med"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"1443","DOI":"10.1056\/NEJMoa051469","article-title":"Genetic modifiers of lung disease in cystic fibrosis","volume":"353","author":"ML Drumm","year":"2005","journal-title":"N Engl J Med"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"344","DOI":"10.1001\/archpedi.1938.01980140114013","article-title":"Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathological study","volume":"56","author":"DH Andersen","year":"1938","journal-title":"Am J Dis Child"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"754","DOI":"10.1378\/chest.125.2.754","article-title":"The role of transforming growth factor beta in lung development and disease","volume":"125","author":"U Bartram","year":"2004","journal-title":"Chest"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"2228","DOI":"10.1093\/hmg\/ddn123","article-title":"Interaction between a anovel TGFB1 haplotype and CFTR genotype is associated with improve lung function in cystic fibrosis","volume":"17","author":"BS Bremer LA","year":"2008","journal-title":"Hum Mol Genet"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1001\/jama.299.4.417","article-title":"Interactions between secondhand smoke and genes that affect cystic fibrosis lung disease","volume":"299","author":"JM Collaco","year":"2008","journal-title":"JAMA"},{"key":"ref12","first-page":"1040","article-title":"Complex two-gene modulation of lung disease severity in children with cystic fibrosis","volume":"118","author":"R Dorfman","year":"2008","journal-title":"J Clin Invest"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"348","DOI":"10.1038\/sj.icb.7100044","article-title":"The regulatory role of TGF-beta in airway remodeling in asthma","volume":"85","author":"T Makinde","year":"2007","journal-title":"Immunol Cell Biol"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"3","DOI":"10.2353\/ajpath.2009.081170","article-title":"Emerging concepts in the pathogenesis of lung fibrosis","volume":"175","author":"WD Hardie","year":"2009","journal-title":"Am J Pathol"},{"key":"ref15","article-title":"Therapeutic Targets in Liver Fibrosis","author":"JA Fallowfield","year":"2011","journal-title":"Am J Physiol Gastrointest Liver Physiol"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"78","DOI":"10.3410\/B2-78","article-title":"Fibrosis: recent advances in myofibroblast biology and new therapeutic perspectives","volume":"2","author":"B Hinz","year":"2010","journal-title":"F1000 Biol Rep"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"L86","DOI":"10.1152\/ajplung.00534.2007","article-title":"Transforming growth factor-\u03b2 signaling mediates hypoxia-induced pulmonary arterial remodeling and inhibition of alveolar development in newborn mouse lung","volume":"295","author":"N Ambalavanan","year":"2008","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"753","DOI":"10.5858\/2009-0230-OA.1","article-title":"Characterization of CXCR4 expression in chondrosarcoma of bone","volume":"135","author":"S Bai","year":"2011","journal-title":"Arch Pathol Lab Med"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"1807","DOI":"10.2353\/ajpath.2007.070112","article-title":"The myofibroblast: one function, multiple origins","volume":"170","author":"B Hinz","year":"2007","journal-title":"Am J Pathol"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"1339","DOI":"10.1084\/jem.20110551","article-title":"Integrating mechanisms of pulmonary fibrosis","volume":"208","author":"TA Wynn","year":"2011","journal-title":"J Exp Med"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"136","DOI":"10.7326\/0003-4819-134-2-200101160-00015","article-title":"Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy","volume":"134","author":"M Selman","year":"2001","journal-title":"Ann Intern Med"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"849","DOI":"10.1042\/BST0370849","article-title":"Role of integrin-mediated TGFbeta activation in the pathogenesis of pulmonary fibrosis","volume":"37","author":"A Goodwin","year":"2009","journal-title":"Biochem Soc Trans"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1016\/j.jcf.2010.03.001","article-title":"Alveolar inflammation in cystic fibrosis","volume":"9","author":"M Ulrich","year":"2010","journal-title":"J Cyst Fibros"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"705","DOI":"10.1016\/j.bbrc.2013.03.032","article-title":"Ceramide mediates lung fibrosis in cystic fibrosis","volume":"434","author":"R Ziobro","year":"2013","journal-title":"Biochem Biophys Res Commun"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.1002\/ppul.21079","article-title":"Transforming growth factor-beta(1) in bronchoalveolar lavage fluid from children with cystic fibrosis","volume":"44","author":"WT Harris","year":"2009","journal-title":"Pediatr Pulmonol"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1002\/ppul.21430","article-title":"Plasma TGF-beta(1) in pediatric cystic fibrosis: Potential biomarker of lung disease and response to therapy","volume":"46","author":"WT Harris","year":"2011","journal-title":"Pediatr Pulmonol"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"1350","DOI":"10.1056\/NEJM200005043421807","article-title":"Role of transforming growth factor beta in human disease","volume":"342","author":"GC Blobe","year":"2000","journal-title":"N Engl J Med"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"L86","DOI":"10.1152\/ajplung.00534.2007","article-title":"Transforming growth factor-beta signaling mediates hypoxia-induced pulmonary arterial remodeling and inhibition of alveolar development in newborn mouse lung","volume":"295","author":"N Ambalavanan","year":"2008","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"L125","DOI":"10.1152\/ajplung.00074.2011","article-title":"Hypoxia-induced inhibition of lung development is attenuated by the peroxisome proliferator-activated receptor-gamma agonist rosiglitazone","volume":"301","author":"T Nicola","year":"2011","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"930","DOI":"10.1136\/thorax.57.11.930","article-title":"Matrix metalloproteases in BAL fluid of patients with cystic fibrosis and their modulation by treatment with dornase alpha","volume":"57","author":"F Ratjen","year":"2002","journal-title":"Thorax"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"721","DOI":"10.1183\/09031936.00173210","article-title":"The role of matrix metalloproteinases in cystic fibrosis lung disease","volume":"38","author":"A Gaggar","year":"2011","journal-title":"Eur Respir J"},{"key":"ref32","doi-asserted-by":"crossref","first-page":"1074","DOI":"10.1136\/thx.2006.074641","article-title":"Airway remodelling in children with cystic fibrosis","volume":"62","author":"TN Hilliard","year":"2007","journal-title":"Thorax"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"1311","DOI":"10.1083\/jcb.200704042","article-title":"Myofibroblast contraction activates latent TGF-beta1 from the extracellular matrix","volume":"179","author":"PJ Wipff","year":"2007","journal-title":"J Cell Biol"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1183\/09031936.03.00006603","article-title":"Progressive damage on high resolution computed tomography despite stable lung function in cystic fibrosis","volume":"23","author":"PA de Jong","year":"2004","journal-title":"Eur Respir J"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"2327","DOI":"10.1378\/chest.128.4.2327","article-title":"Dornase alfa reduces air trapping in children with mild cystic fibrosis lung disease: a quantitative analysis","volume":"128","author":"TE Robinson","year":"2005","journal-title":"Chest"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1183\/09059180.00001111","article-title":"Antifibrotic activities of pirfenidone in animal models","volume":"20","author":"CJ Schaefer","year":"2011","journal-title":"Eur Respir Rev"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"1589","DOI":"10.1084\/jem.20100035","article-title":"miR-21 mediates fibrogenic activation of pulmonary fibroblasts and lung fibrosis","volume":"207","author":"G Liu","year":"2010","journal-title":"J Exp Med"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"1760","DOI":"10.1016\/S0140-6736(11)60405-4","article-title":"Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials","volume":"377","author":"PW Noble","year":"2011","journal-title":"Lancet"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"56","DOI":"10.1164\/rccm.200706-805OC","article-title":"Partial inhibition of integrin alpha<sub>v<\/sub>\u03b26 prevents pulmonary fibrosis without exacerbating inflammation","volume":"177","author":"GS Horan","year":"2008","journal-title":"Am J Respir Crit Care Med"},{"key":"ref40","doi-asserted-by":"crossref","first-page":"82","DOI":"10.1164\/rccm.200706-806OC","article-title":"Inhibition of integrin alpha(v)beta6, an activator of latent transforming growth factor-beta, prevents radiation-induced lung fibrosis","volume":"177","author":"K Puthawala","year":"2008","journal-title":"Am J Respir Crit Care Med"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"208219","DOI":"10.1155\/2011\/208219","article-title":"Blocking TGFbeta via Inhibition of the alphavbeta6 Integrin: A Possible Therapy for Systemic Sclerosis Interstitial Lung Disease","volume":"2011","author":"TR Katsumoto","year":"2011","journal-title":"Int J Rheumatol"},{"key":"ref42","doi-asserted-by":"crossref","first-page":"1079","DOI":"10.1056\/NEJMoa1103690","article-title":"Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis","volume":"365","author":"L Richeldi","year":"2011","journal-title":"N Engl J Med"},{"key":"ref43","doi-asserted-by":"crossref","first-page":"159","DOI":"10.7326\/0003-4819-152-3-201002020-00007","article-title":"Narrative review: fibrotic diseases: cellular and molecular mechanisms and novel therapies","volume":"152","author":"J Rosenbloom","year":"2010","journal-title":"Ann Intern Med"}],"updated-by":[{"DOI":"10.1371\/annotation\/d0132de3-56ca-4258-9119-bdab0ceb2cff","type":"correction","label":"Correction","source":"publisher","updated":{"date-parts":[[2013,8,29]],"date-time":"2013-08-29T00:00:00Z","timestamp":1377734400000}}],"container-title":["PLoS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0070196","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,3,3]],"date-time":"2022-03-03T16:47:12Z","timestamp":1646326032000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0070196"}},"subtitle":[],"editor":[{"given":"Rory Edward","family":"Morty","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2013,8,12]]},"references-count":43,"journal-issue":{"issue":"8","published-online":{"date-parts":[[2013,8,12]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0070196","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,8,12]]}}}