Restoration of impaired endothelial myocyte enhancer factor 2 function rescues pulmonary arterial hypertension.

ABSTRACT:

Background

Pulmonary arterial hypertension (PAH) is a progressive disease of the pulmonary arterioles, characterized by increased pulmonary arterial pressure and right ventricular failure. The cause of PAH is complex, but aberrant proliferation of the pulmonary artery endothelial cells (PAECs) and pulmonary artery smooth muscle cells is thought to play an important role in its pathogenesis. Understanding the mechanisms of transcriptional gene regulation involved in pulmonary vascular homeostasis can provide key insights into potential therapeutic strategies.

Methods and results

We demonstrate that the activity of the transcription factor myocyte enhancer factor 2 (MEF2) is significantly impaired in the PAECs derived from subjects with PAH. We identified MEF2 as the key cis-a

SUBMITTER: Kim J 

PROVIDER: S-EPMC4293354 | biostudies-literature | 2015 Jan

REPOSITORIES: biostudies-literature