- 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
- 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
Scientists may have found a
therapy to stop the deadly and mostly untreatable lung disease, idiopathic pulmonary
(IPF), according to the preclinical data of the new study.
‘New molecular target identified for treating deadly lung disease called idiopathic pulmonary fibrosis.’
"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 FibrosisPulmonary 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
- A gene called FOXF1 inhibits the progression of idiopathic
- 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.
- Study Identifies New Molecular Target for Treating Deadly Lung Disease IPF - (https://www.cincinnatichildrens.org/news/release/2018/idiopathic-pulmonary-fibrosis)