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New finding could lead to cure of pulmonary arterial hypertension

(Xinhua)
Updated: 2009-10-26 10:24

LOS ANGELES: A groundbreaking discovery has been made by US researchers that may lead to future drug therapies to extend the life of patients with pulmonary arterial hypertension.

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Researchers at the University of California, San Diego (UCSD) have identified a key protein that promotes the development of pulmonary arterial hypertension in humans and mice.

The finding was published in a paper online in Nature Medicine on Sunday.

The new discovery would make it possible for scientists to develop a kind of drugs to prevent the need for lung transplantation for pulmonary arterial hypertension patients. Tillnow the only cure for this debilitating disease is to have lung transplantation.

According to Patricia Thistlethwaite, Professor of Surgery and cardiothoracic surgeon in UCSD's Department of Surgery and the research team, vascular smooth muscle cells associated with pulmonary arterial hypertension are switched on to proliferate by a receptor protein called Notch-3.

Researchers, with this finding, were able to block and reverse the pathway of disease in mice.

"The UCSD team found that pulmonary hypertension is characterized by over-expression of Notch-3 and that the severity of the disease correlates with the amount of this protein in the lung," said Thistelthwaite.

"We showed that a mouse model lacking this protein does not develop pulmonary hypertension, and in addition, that the disease can be effectively treated with an enzyme called Y-secretase inhibitor, which blocks Notch-3 activation," Thistelthwaite added.

Researchers found that mice with pulmonary arterial hypertension that were treated with the Y-secretase inhibitor showed reversal of the disease.

It is reported that forms of this drug are currently in use in Phase 1 trials for the treatment of Alzheimer's disease.

Pulmonary arterial hypertension is a form of high blood pressure in the lung's arteries. About 100,000 Americans have the problem, causing 20,000 deaths every year.