endothelin-1 and Channelopathies

Pulmonary arterial hypertension (PAH) is a devastating disease with high morbidity and mortality. Deleterious remodeling in the pulmonary arterial system leads to irreversible arterial constriction and elevated pulmonary arterial pressures, right heart failure, and eventually death. The difficulty in treating PAH stems in part from the complex nature of disease pathogenesis, with several signaling compounds known to be involved (e.g., endothelin-1, prostacyclins) which are indeed targets of PAH therapy. Over the last decade, potassium channelopathies were established as novel causes of PAH. More specifically, loss-of-function mutations in the

Topics: Channelopathies; Endothelin-1; Familial Primary Pulmonary Hypertension; Humans; Hypertension, Pulmonary; KATP Channels; Nerve Tissue Proteins; Potassium; Potassium Channels, Tandem Pore Domain; Potassium Channels, Voltage-Gated; Prostaglandins I; Pulmonary Arterial Hypertension