Dr. Harry Karmouty-Quintana
The University of Texas Health Science Center at Houston
McGovern Medical School
Department of Biochemistry and Molecular Biology
Pulmonary hypertension (PH) is a disorder of the pulmonary vasculature defined by increased mean pulmonary arterial pressure leading to right ventricle hypertrophy, right-sided heart failure and ultimately death. PH is a common complication in chronic lung diseases, currently the 3rd killer in the USA, where it is strongly associated with increased morbidity and mortality. The pathologic process in PH is characterized by extensive vascular remodeling; including enhanced proliferation of pulmonary artery smooth muscle cells (PASMC) leading to narrowing and obliteration of the vessel lumen resulting in increased vascular tone. However, little is known regarding the mechanisms that govern vascular remodeling leading to PH in IPF and as a result, effective treatment options are lacking.
The focus of our laboratory is to use experimental models of disease together with primary cell culture techniques and human lung tissue from patients with ongoing disease to study the mechanisms that lead to PH secondary to chronic lung disease. Currently, we are focusing in the role extracellular matrix remodeling, specifically the role of hyaluronan, a pluripotent glycosaminoglycan, in the development of PH in fibrotic lung diseases where excess tissue remodeling is prevalent.