Finding cellular causes of lung-hardening disease

September 17, 2013 by Tracey Peake, North Carolina State University
Finding cellular causes of lung-hardening disease

(Medical Xpress)—Idiopathic Pulmonary Fibrosis, or IPF, is an incurable lung disease that, over time, turns healthy lung tissue into inflexible scar tissue – hardening the lungs and eventually causing respiratory distress and death. Currently, there is no cure.

Phil Sannes, a professor of cell biology, studies IPF on the cellular level. In his most recent research, he's found that in the case of IPF patients, three growth factors within different types of cells in the lung may be working together to cause the disease.

Previous research, including earlier studies from Sannes' lab, established two signaling molecules that seemed to be involved in the development of IPF: Wnt7B and TGF-?. In normal lungs, Wnt7B regulates the epithelial cells on the surface of the lung, and TGF-? plays a role in the development of fibroblasts in the layers underneath the epithelium. When the lung is damaged, Wnt7B and TGF-? help cells in the lung work together to make sure that the injured area is sealed off and the epithelium can repair itself. If the epithelium can't recover within roughly 48 hours after the injury, then fibroblast production accelerates and the quick growing fibroblasts create a scar. When the recovers, fibroblast production gets turned off and the scar eventually resolves itself.

In IPF, fibroblast production is stuck in overdrive. Somehow, the don't get the message to stop producing. Sannes' latest work, supported by the NIH, identifies a third possible player in the mix, a signaling molecule known as fibroblast growth factor nine (FGF9). In a paper in the Journal of Histochemistry and Cytochemistry, Sannes shows that FGF9, which is normally found only in the smooth muscle tissue in the lungs, also appears in epithelial cells of IPF patients.

"FGF9 is important to , but in normal lungs it's only found in small amounts in smooth muscle tissue," Sannes says. "In fact, none of the three molecules are expressed a lot in normal lung – just when they're needed in the healing process.

"Finding FGF9 out of place like this is unusual. We don't know why it's there or what its role is, but our operating hypothesis is that in an IPF lung FGF9, Wnt7B and TGF-? are acting together in a coordinated way. Our next steps will be to find out how this combination may lead to development of IPF."

Explore further: New insights come from tracing cells that irreversibly scar lungs

More information: jhc.sagepub.com/content/61/9/671.full

Related Stories

New insights come from tracing cells that irreversibly scar lungs

December 1, 2011
Idiopathic Pulmonary Fibrosis (IPF) is an incurable disease in which the delicate gas exchange region of the lung fills with scar tissue, which interferes with breathing. Now researchers at Duke University Medical Center ...

Metabolomics key to identifying disease pathway: Research reveals lactic acid's role in lung disease

January 14, 2013
(Medical Xpress)—Expertise at Pacific Northwest National Laboratory contributed to the understanding of the role of cellular metabolism in the pathogenesis of a currently untreatable lung disease. This research, reported ...

Chinese herbs show promise for lung cancer, flu, and idiopathic pulmonary fibrosis

October 22, 2012
Chinese herbs, including JHQG, BFXL, and BFHX, may show significant benefits for patients with non-small cell lung cancer (NSCLC), idiopathic pulmonary fibrosis (IPF), and influenza.

Recommended for you

Scientists emulate the human blood-retinal barrier on a microfluidic chip

January 24, 2018
For some years, scientists have been seeking ways to reduce animal testing and accelerate clinical trials. In vitro assays with living cells are an alternative, but have limitations, as the interconnection and interaction ...

Forces from fluid in the developing lung play an essential role in organ development

January 23, 2018
It is a marvel of nature: during gestation, multiple tissue types cooperate in building the elegantly functional structures of organs, from the brain's folds to the heart's multiple chambers. A recent study by Princeton researchers ...

Anemia discovery offers new targets to treat fatigue in millions

January 22, 2018
A new discovery from the University of Virginia School of Medicine has revealed an unknown clockwork mechanism within the body that controls the creation of oxygen-carrying red blood cells. The finding sheds light on iron-restricted ...

More surprises about blood development—and a possible lead for making lymphocytes

January 22, 2018
Hematopoietic stem cells (HSCs) have long been regarded as the granddaddy of all blood cells. After we are born, these multipotent cells give rise to all our cell lineages: lymphoid, myeloid and erythroid cells. Hematologists ...

How metal scaffolds enhance the bone healing process

January 22, 2018
A new study shows how mechanically optimized constructs known as titanium-mesh scaffolds can optimize bone regeneration. The induction of bone regeneration is of importance when treating large bone defects. As demonstrated ...

Researchers illustrate how muscle growth inhibitor is activated, could aid in treating ALS

January 19, 2018
Researchers at the University of Cincinnati (UC) College of Medicine are part of an international team that has identified how the inactive or latent form of GDF8, a signaling protein also known as myostatin responsible for ...

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.