Gene Activity Analysis of IPF Lung Cells Pinpoints Abnormalities, Opens Way to Targeted Treatments

Gene Activity Analysis of IPF Lung Cells Pinpoints Abnormalities, Opens Way to Targeted Treatments

Wide access to potential molecular drug targets to treat pulmonary fibrosis is now possible, as researchers have mapped the complete set of genes active in individual lung cells from patients with idiopathic lung fibrosis and from healthy people.

The analysis, called single-cell RNA sequencing, also made it possible to single out several abnormal molecular pathways in the cells.

The study, “Single-cell RNA sequencing identifies diverse roles of epithelial cells in idiopathic pulmonary fibrosis,” published in the Journal of Clinical Investigation Insights, is likely to open up focused research on the molecular processes thought to contribute to the scarring of lung tissue.

Many studies have attempted to identify such drug targets. But using older methods, researchers were only able to detect changes in samples of whole tissue.

Now, researchers at Cincinnati Children’s Hospital Medical Center and Cedars-Sinai Medical Center in Los Angeles analyzed gene activity profiles from individual epithelial cells — the cell type lining the inside of the lungs. The cells were acquired either by brushing the airways or from tissue biopsies.

This approach allowed them to pinpoint the existence of different types of epithelial cells in healthy and diseased lungs. In healthy lungs, they found plenty of cells known as alveolar type 2 cells, which make sure that the inside surface of the lungs is moist. These cells are critical, both to normal breathing and to keeping microbes at bay.

Epithelial cells from idiopathic pulmonary fibrosis (IPF) patients could be divided into three types. While two were cell types also seen in healthy people, the third was nothing similar to what is found in normal lungs. In contrast to healthy cells, which usually have a specialized function, the abnormal cells had characteristics of several cell types — a feature shared with tumor cells. These cells are the ones driving disease processes.

The team identified abnormal activation of several well-known molecular signaling cascades in the diseased cells, and microscopic analysis confirmed that the cells had lost the normal appearance seen in lung alveoli.

“This paper identifies a number of novel targets and molecular pathways for IPF, for which there are pharmaceutical approaches,” Jeffrey Whitsett, MD, lead investigator and co-director of the Perinatal Institute at Cincinnati Children’s, said in a news release.

11 comments

  1. Denise says:

    Oooo, keep it up, we need a cure, or at least what causes it so it can be prevented! Please, please, please
    Thank you for posting the progression of these studies. It gives us hope.
    Does this pertain to all lung fibrosis or just IPF.

    • Magdalena Kegel says:

      Dear Denise,

      They only analyzed cells of IPF patients so it is currently impossible to say if similar changes are present in other patients.

  2. Valton Douglas says:

    There has been three, possibly four, deaths in our family, from this decease, two presently dealing with this decease, one recovering from double lung transplant and many anxious about their risk of being found with this signature of death!!! May God help find the cure!

    • Tim Bossie says:

      Wow Valton, we are truly sorry for your loss and the pain that this horrible disease has brought to your family. We, along with you, are hoping for a cure to be found quickly.

  3. Melissa Lingefelt says:

    My husbands mother died of this disease and my husband just had a biopsy done. If he does have it can we try this trail?

  4. Lee Seidman says:

    recent diagnosis as follows after Lung Biopsy at the Cleveland clinic. Please alert me to any trials or progress relating to treatment. leeseidman@aol.com

    “Two entlties come to mind, one is a predominant fibrotic
    phase
    of NSIP and alternatively, chronic hypersensitivlty pneumonitis that
    perhaps it is in a burned out stage.”

  5. Kathy says:

    I was diagnosed in 2013 with IPF. I pray for a cure everyday but I am living a quality life. Lots to do, with loving friends and family. Being put on IPF drug today, so hope it slows things down.

    • Tim Bossie says:

      Hi Kathy! It is great to hear that you are doing well and that you are starting on a new treatment. Would you mind sharing what that is with the rest of our readers?

  6. Andrea says:

    My grandmother, mother three of her sisters have all died from this.2 of her sisters are still living with it. My sister and I both have scar tissue forming in our lungs now.

  7. Sara A says:

    If diagnosed how can we get in contact tact with you? Is there a waiting list? Is it easy to see you in Cincinnati or LA? Since it’s hereditary should we be tested to see if we carry the gene?

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