New Molecular Target to Treat Deadly Lung Disease

Highlights:

A novel anti-fibrotic drug target that inhibits pulmonary fibrosis was identified by a new study
A gene called FOXF1 inhibits the progression of pulmonary fibrosis which includes excessive scarring of lung tissue and inflammation.
Therapeutic tools to boost FOXF1 levels in idiopathic pulmonary fibrosis patients could be an effective therapy to treat the disease.

A gene called FOXF1 inhibits the progression of idiopathic pulmonary fibrosis (IPF) which includes extensive scarring in lung tissues, hyper-production of harmful cells called myofibroblasts and excessive lung inflammation, shows new study. The study was conducted by a research team from the Cincinnati Children's Hospital Medical Center and published in the journalCell Reports.

Scientists may have found a therapy to stop the deadly and mostly untreatable lung disease, idiopathic pulmonary fibrosis(IPF), according to the preclinical data of the new study.

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‘New molecular target identified for treating deadly lung disease called idiopathic pulmonary fibrosis.’

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"The exact cause of IPF is unknown and effective treatments are needed. This study identifies a novel anti-fibrotic drug target that inhibits pulmonary fibrosis in our preclinical models," said lead investigator Tanya Kalin, MD, PhD, Division of Pulmonary Biology. "We are developing different therapeutic approaches and conducting preclinical tests to increase FOXF1 expression in the cells of lung connective tissues."

Idiopathic Pulmonary Fibrosis

Pulmonary fibrosis is a condition where the lung tissue gets scarred making it difficult for the patient to breathe. The cause of idiopathic pulmonary fibrosis remains unknown but genetic predisposition, smoking and other potential environmental factors are suspected. The most common symptoms of the disease include shortness of breath and a persistent dry, hacking cough. It usually affects people between the ages of 50 and 70, but it can strike younger adults and children, according to the National Library of Medicine at the National Institutes of Health (NIH). Around 100,000 people are affected in the United States, with an estimated 30,000 to 40,000 new cases diagnosed annually.

Study findings

A gene called FOXF1 inhibits the progression of idiopathic pulmonary fibrosis.
Human lungs from IPF patients and mouse models of IPF lack FOXF1 in myofibroblasts.
Cells lacking FOXF1 exhibited overexpression of a related gene called FOXM1, which drives lung scarring and inflammation.

While the data has the potential to develop novel treatment for the disease, the team estimates two to three years of additional laboratory study and development before the data can be applied to clinical treatment.

The team is working on therapeutic tools to boost FOXF1 levels in IPF patients and is currently testing a novel small-molecule compound that stabilizes FOXF1 and inhibits myofibroblasts.

Reference:

Study Identifies New Molecular Target for Treating Deadly Lung Disease IPF - (https://www.cincinnatichildrens.org/news/release/2018/idiopathic-pulmonary-fibrosis)

Source-Medindia

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