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      Idiopathic Pulmonary Fibrosis: Aberrant Recapitulation of Developmental Programs?

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      Author(s): * , ,  
      Publication date (Electronic):
      Journal: PLoS Medicine
      Publisher: Public Library of Science

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          Abstract

          The authors discuss evidence suggesting that embryonic signaling pathways involved in epithelium/mesenchymal communication and epithelial cell plasticity may be aberrantly switched on in idiopathic pulmonary fibrosis.

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          Most cited references62

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          Idiopathic pulmonary fibrosis: prevailing and evolving hypotheses about its pathogenesis and implications for therapy.

          M. Selman, T E King, A. Pardo (2001)
          Idiopathic pulmonary fibrosis is a progressive and usually fatal lung disease characterized by fibroblast proliferation and extracellular matrix remodeling, which result in irreversible distortion of the lung's architecture. Although the pathogenetic mechanisms remain to be determined, the prevailing hypothesis holds that fibrosis is preceded and provoked by a chronic inflammatory process that injures the lung and modulates lung fibrogenesis, leading to the end-stage fibrotic scar. However, there is little evidence that inflammation is prominent in early disease, and it is unclear whether inflammation is relevant to the development of the fibrotic process. Evidence suggests that inflammation does not play a pivotal role. Inflammation is not a prominent histopathologic finding, and epithelial injury in the absence of ongoing inflammation is sufficient to stimulate the development of fibrosis. In addition, the inflammatory response to a lung fibrogenic insult is not necessarily related to the fibrotic response. Clinical measurements of inflammation fail to correlate with stage or outcome, and potent anti-inflammatory therapy does not improve outcome. This review presents a growing body of evidence suggesting that idiopathic pulmonary fibrosis involves abnormal wound healing in response to multiple, microscopic sites of ongoing alveolar epithelial injury and activation associated with the formation of patchy fibroblast-myofibroblast foci, which evolve to fibrosis. Progress in understanding the fibrogenic mechanisms in the lung is likely to yield more effective therapies.
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            Telomerase mutations in families with idiopathic pulmonary fibrosis.

            Mary Armanios, Julian J L Chen, Joy Cogan … (2007)
            Idiopathic pulmonary fibrosis is progressive and often fatal; causes of familial clustering of the disease are unknown. Germ-line mutations in the genes hTERT and hTR, encoding telomerase reverse transcriptase and telomerase RNA, respectively, cause autosomal dominant dyskeratosis congenita, a rare hereditary disorder associated with premature death from aplastic anemia and pulmonary fibrosis. To test the hypothesis that familial idiopathic pulmonary fibrosis may be caused by short telomeres, we screened 73 probands from the Vanderbilt Familial Pulmonary Fibrosis Registry for mutations in hTERT and hTR. Six probands (8%) had heterozygous mutations in hTERT or hTR; mutant telomerase resulted in short telomeres. Asymptomatic subjects with mutant telomerase also had short telomeres, suggesting that they may be at risk for the disease. We did not identify any of the classic features of dyskeratosis congenita in five of the six families. Mutations in the genes encoding telomerase components can appear as familial idiopathic pulmonary fibrosis. Our findings support the idea that pathways leading to telomere shortening are involved in the pathogenesis of this disease. Copyright 2007 Massachusetts Medical Society.
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              BMP-7 counteracts TGF-beta1-induced epithelial-to-mesenchymal transition and reverses chronic renal injury.

              Michael Zeisberg, Jun-ichi Hanai, Hikaru Sugimoto … (2003)
              Bone morphogenic protein (BMP)-7 is a 35-kDa homodimeric protein and a member of the transforming growth factor (TGF)-beta superfamily. BMP-7 expression is highest in the kidney, and its genetic deletion in mice leads to severe impairment of eye, skeletal and kidney development. Here we report that BMP-7 reverses TGF-beta1-induced epithelial-to-mesenchymal transition (EMT) by reinduction of E-cadherin, a key epithelial cell adhesion molecule. Additionally, we provide molecular evidence for Smad-dependent reversal of TGF-beta1-induced EMT by BMP-7 in renal tubular epithelial cells and mammary ductal epithelial cells. In the kidney, EMT-induced accumulation of myofibroblasts and subsequent tubular atrophy are considered key determinants of renal fibrosis during chronic renal injury. We therefore tested the potential of BMP-7 to reverse TGF-beta1-induced de novo EMT in a mouse model of chronic renal injury. Our results show that systemic administration of recombinant human BMP-7 leads to repair of severely damaged renal tubular epithelial cells, in association with reversal of chronic renal injury. Collectively, these results provide evidence of cross talk between BMP-7 and TGF-beta1 in the regulation of EMT in health and disease.
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                Author and article information

                Journal
                Journal ID (nlm-ta): PLoS Med
                Journal ID (publisher-id): pmed
                Journal ID (publisher-id): plme
                Journal ID (pmc): plosmed
                Title: PLoS Medicine
                Publisher: Public Library of Science (San Francisco, USA )
                ISSN (Print): 1549-1277
                ISSN (Electronic): 1549-1676
                Publication date (Print): March 2008
                Publication date (Electronic): 4 March 2008
                Volume: 5
                Issue: 3
                Electronic Location Identifier: e62
                Author notes
                * To whom correspondence should be addressed. E-mail: moiselman@ 123456salud.gob.mx ; mselmanl@ 123456yahoo.com.mx
                Article
                Publisher ID: 07-PLME-RIT-1075R2
                DOI: 10.1371/journal.pmed.0050062
                PMC ID: 2265304
                PubMed ID: 18318599
                SO-VID: d631cc96-902b-49c8-9050-ebffeff00906
                Copyright © Copyright: © 2008 Selman et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
                History
                Page count
                Pages: 8
                Categories
                Subject: Research in Translation
                Subject: Developmental Biology
                Subject: Genetics and Genomics
                Subject: Respiratory Medicine
                Subject: Respiratory Medicine
                Subject: Interstitial Lung Disease
                Subject: Genetics
                Custom metadata
                citation Selman M, Pardo A, Kaminski N (2008) Idiopathic pulmonary fibrosis: Aberrant recapitulation of developmental programs? PLoS Med 5(3): e62. doi: 10.1371/journal.pmed.0050062

                ScienceOpen disciplines: Medicine
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                ScienceOpen disciplines: Medicine

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