Scientists have discovered a new type of cell that is key to the development of pulmonary hypertension, and they believe it may lead to new targets for improved treatments.

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The researchers show how a single progenitor cell identified in normal lung blood vessels migrates and multiplies in pulmonary hypertension.
Image credit: Yale University

Pulmonary hypertension is a rare, debilitating disease where high blood pressure in the arteries of the lungs can lead to right heart failure.

Symptoms include breathlessness, fatigue and dizziness.

Although treatments to manage the disease exist, around half of patients die within 3 years of diagnosis.

A principal biological feature of pulmonary hypertension is that normally unmuscularized tissue becomes muscularized as smooth muscle cells invade various regions of the blood vessels.

However, the underlying events that happen in and around cells to make this happen is poorly understood.

In the new study, led by Yale University in New Haven, CT, scientists gain further insights after studying human lung samples and a mouse model of pulmonary hypertension.

They discovered specialized cells called progenitor cells that reside in the smooth muscle of normal lung blood vessels migrate and multiply in pulmonary hypertension.

Senior author Daniel M. Greif, assistant professor of cardiology at Yale University School of Medicine, says they find it “remarkable that these progenitor cells are present in the normal lung – almost sitting there poised to multiply and migrate in disease.”

He and his colleagues also discovered the molecular signals that regulate the progenitor cells. These signals include the activity of a protein called KLF4, which appears to play a key role in helping smooth muscle cells migrate and “muscularize.”

The team now plans to study the progenitor cells further and classify them so new therapies can be developed to help patients with pulmonary hypertension, for whom there are currently few treatment options, as Prof. Greif explains:

If a patient is diagnosed early, and these cells could be targeted and manipulated, we could potentially attenuate the course of the patient’s disease.”

Doctors are usually reluctant to advise patients with pulmonary hypertension to exercise, because they fear it might place too much strain on the heart. However, Medical News Today recently learned about a new review that suggests patients with pulmonary hypertension can safely benefit from exercise and improve their quality of life.