Collaborative Study Provides Increased Understanding of Idiopathic Pulmonary Fibrosis

A study conducted by researchers at Nagoya University in Japan and researchers from Yale University in the United States was recently published in the European Respiratory Journal. 

This study utilized single-cell RNA sequencing and found a subset of cells that may be able to protect from idiopathic pulmonary fibrosis (IPF). This could be the beginning of a new therapy for the rare disease.


IPF is a rare condition with a very poor prognosis. There is currently a 5-year survival rate.

Researchers don’t know what causes the disease, which leads scar tissue to build up in the lungs. This impairs the ability of the lungs to function as they should and properly exchange gas. As a result, patients face breathing issues.

Unfortunately, there is currently no cure for the disease. However, this new study may lead to improved research toward a potential treatment.

The Study

This investigation studied lung tissue of those with IPF and those with normal lungs. A total of 250,000 cells from lung tissues were included. They came from 32 lungs with IPF and 29 lungs which were normal. Each cell was sequenced individually. This allowed the team to see which genes were expressed in each cell.

They found that there was one subset of cells that were more common in the IPF lungs. These were fibroblast cells, which are the most common within cells’ supportive tissue and surrounding organs. These fibroblast cells produced Meflin, which is a protein.

These cells were most common in acute focal lesions within the lung where there was very dense scarring.

When the team used mouse models of IPF and turned off Meflin, the cells aged, and the IPF was much worse than what would have been expected without the turn-off of Meflin.

The next steps are to further investigate how Meflin is able to protect lung tissue from fibrosis. Meflin cells may be able to treat IPF. Further, they may be used as a part of its diagnosis.

Ultimately, this study helps us better understand IPF and its mechanisms. The hope is that it will eventually lead to a new therapy.

You can read more about this study and its findings here.

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