Harry Karmouty-Quintana, Ph.D.

Pulmonary hypertension (PH) is a common complication of chronic obstructive pulmonary disease (COPD) that can worsen the chronic lung disease and impact patient survival. One of the main components of PH is the excessive multiplication of pulmonary smooth muscle cells, which line the lungs' blood vessels. These cells multiply in an uncontrolled manner, causing lesions in the arteries and veins of the lung that lead to obstruction of blood flow and increased blood pressure in the lungs. This causes the right side of the heart to over work, eventually leading to heart attacks and death. We will study why these cells multiply uncontrollably. Our findings could lead to novel therapies to treat PH in COPD that could prolong life expectancy.

Update: The focus of our project is to understand the role of CFIm25, a protein that is reduced in patients with COPD who have high blood pressure in the lungs. Our data show that in patients with COPD, reduced levels of CFIm25 are apparent. These changes are consistent with increased expression of an enzyme called hyaluronan synthase 2 (HAS2). This enzyme t is responsible for enhanced synthesis of a substance associated with increased scarring and the development of pulmonary hypertension. Preliminary data from our lab showed that treatment with a drug that targets hyaluronan production leads to reduced PH and improved markers of chronic lung injury. Our results have provided us with the rationale to use an FDA-approved drug against HAS2 that has shown to reduce pulmonary hypertension and markers of chronic lung injury in mice.