Future Directions in Genetic Strategies for Understanding and Treating Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis (IPF) is a type of chronic, progressive lung disease with unknown cause, which is characterized by increasing dyspnea and destruction of lung function with a high mortality rate. Evolving evidence demonstrated that the pathogenesis of IPF involved multiple signaling pathways such as inflammation, oxidative stress and fibrosis. However, drug discovery to prevent or revert IPF has been insufficient to cope with the development. Drug discovery targeting multiple links should be considered. In this review, we will brief the pathogenesis of IPF and discuss several small chemical entities toward the pathogenesis for IPF studied in animal models and clinical trials. The field of novel anti-IPF agents and the future directions for the prevention and treatment of IPF are detailed thoroughly discussed.

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Epigenetics in idiopathic pulmonary fibrosis

Biochemistry and Cell Biology ◽

10.1139/bcb-2014-0126 ◽

2015 ◽

Vol 93 (2) ◽

pp. 159-170 ◽

Cited By ~ 45

Author(s):

Argyrios Tzouvelekis ◽

Naftali Kaminski

Keyword(s):

Lung Cancer ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Wound Repair ◽

Environmental Changes ◽

Research Field ◽

Review Article ◽

Lung Disorder ◽

Future Directions ◽

Disease Phenotypes

Idiopathic pulmonary fibrosis (IPF) is a lethal chronic lung disorder with no effective treatment and a prognosis worse than that of lung cancer. Despite extensive research efforts, its etiology and pathogenesis still remain largely unknown. Current experimental evidence has shifted the disease paradigm from chronic inflammation towards the premise of abnormal epithelial wound repair in response to repeated epigenetic injurious stimuli in genetically predisposed individuals. Epigenetics is defined as the study of heritable changes in gene function by factors other than an individual’s DNA sequence, providing valuable information regarding adaption of genes to environmental changes. Although cancer is the most studied disease with relevance to epigenetic modifications, recent data support the idea that epigenomic alterations may lead to variable disease phenotypes, including fibroproliferative lung disorders such as IPF. This review article summarizes the latest experimental and translational epigenetic studies in the research field of chronic lung disorders, mainly focusing on IPF, highlights current methodology limitations, and underlines future directions and perspectives.

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Future Directions in the Treatment of Idiopathic Pulmonary Fibrosis

Lung Biology in Health and Disease - Idiopathic Pulmonary Fibrosis ◽

10.1201/b14211-28 ◽

2003 ◽

pp. 699-732

Author(s):

Kevin Brown ◽

Marvin Schwarz

Keyword(s):

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Future Directions

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