Researchers at the University of Alberta have identified a "central command system" for pulmonary hypertension, a disease that currently has no cure and kills thousands each year.
"We believe this finding opens the door to a new strategy and the development of a new stream of drugs to treat this complex and lethal disease," said Dr. Evangelos Michelakis, director of the U of A pulmonary hypertension program and senior author of the study, which appears today in the Proceedings of the National Academy of Sciences.
Much like cancer, pulmonary hypertension is caused by the uncontrolled proliferation of cells. In the case of pulmonary hypertension, these cells build up in the pulmonary arteries, block blood flow and cause perpetual high-blood pressure in the lungs and, eventually, heart failure.
Pulmonary hypertension is especially dangerous because it is difficult to diagnose, Michelakis said, adding that it can appear in anyone at any age but is most commonly found in females in their 30s. The prime symptom is shortness of breath.
"The current treatments for pulmonary hypertension do not work. At best they might somewhat prolong and improve quality of life, but nothing cures itit usually kills patients within four to five years from diagnosis," said Sebastien Bonnet, a post-doctoral fellow in the U of A Department of Medicine and first author of the paper.
However, the U of A researchers believe they've made a breakthrough in their discovery of the role that nuclear-factor-of-activated-T-lymphocytes (NFAT) play in the cause of the disease. The researchers have identified NFAT as a potential controlling element of pulmonary hypertension in both human tissues and animal models.
When it is working properly, NFAT co-ordinates several cellular functions by regulating the expression of multiple genes. It is normally activated in important health-maintaining functions, such as the regulation of immune cells and t
Contact: Ryan Smith
University of Alberta