Developmental pathways in the pathogenesis of lung fibrosis
| Autor: | Diptiman Chanda, Thomas Volckaert, Stijn De Langhe, Victor J. Thannickal, Samuel R. Smith, Eva Otoupalova |
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| Rok vydání: | 2019 |
| Předmět: |
Pulmonary Fibrosis
Clinical Biochemistry Disease Bioinformatics Biochemistry Article Pulmonary function testing Pathogenesis Idiopathic pulmonary fibrosis Transforming Growth Factor beta Fibrosis Pulmonary fibrosis medicine Genetic predisposition Animals Humans Myofibroblasts Molecular Biology Homeodomain Proteins Lung business.industry General Medicine respiratory system medicine.disease respiratory tract diseases medicine.anatomical_structure Cellular Microenvironment Trans-Activators Molecular Medicine Disease Susceptibility Stromal Cells business Biomarkers Signal Transduction |
| Zdroj: | Molecular Aspects of Medicine. 65:56-69 |
| ISSN: | 0098-2997 |
| Popis: | Idiopathic pulmonary fibrosis (IPF) is a progressive and terminal lung disease with no known cure. IPF is a disease of aging, with median age of diagnosis over 65 years. Median survival is between 3 and 5 years after diagnosis. IPF is characterized primarily by excessive deposition of extracellular matrix (ECM) proteins by activated lung fibroblasts and myofibroblasts, resulting in reduced gas exchange and impaired pulmonary function. Growing evidence supports the concept of a pro-fibrotic environment orchestrated by underlying factors such as genetic predisposition, chronic injury and aging, oxidative stress, and impaired regenerative responses may account for disease development and persistence. Currently, two FDA approved drugs have limited efficacy in the treatment of IPF. Many of the genes and gene networks associated with lung development are induced or activated in IPF. In this review, we analyze current knowledge in the field, gained from both basic and clinical research, to provide new insights into the disease process, and potential approaches to treatment of pulmonary fibrosis. |
| Databáze: | OpenAIRE |
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