Abstract
Pulmonary hypertension is a serious clinical condition characterised by increased pulmonary arterial pressure. This can lead to right ventricular failure which can be fatal. Connexins are gap junction-forming membrane proteins which serve to exchange small molecules of less than 1 kD between cells. Connexins can also form hemi-channels connecting the intracellular and extracellular environments. Hemi-channels can mediate adenosine triphosphate release and are involved in autocrine and paracrine signalling. Recently, our group and others have identified evidence that connexin-mediated signalling may be involved in the pathogenesis of pulmonary hypertension. In this review, we discuss the evidence that dysregulated connexin-mediated signalling is associated with pulmonary hypertension.
Highlights
Pulmonary artery endothelial cells (PAECs), pulmonary artery smooth muscle cells (PASMCs) and pulmonary artery fibroblasts (PAFs) are all involved in the pulmonary vascular remodelling process
The increase in mean pulmonary arterial pressure observed in patients with Pulmonary Hypertension (PH) can be due to a variety of causes, PH can be sub-divided into five main clinical groups (Table 1): pulmonary arterial hypertension (PAH; group 1), PH due to left heart disease, PH due to chronic lung diseases or hypoxia, chronic thromboembolic
In vitro studies showed that the myoendothelial gap junctions (MEJ) between between rat PAECs and PASMCs were primarily formed by Cx43 [27]
Summary
Pulmonary hypertension (PH) is defined by a chronic increase in mean pulmonary arterial pressure [1]. The increase in mean pulmonary arterial pressure observed in patients with PH can be due to a variety of causes, PH can be sub-divided into five main clinical groups (Table 1): pulmonary arterial hypertension (PAH; group 1), PH due to left heart disease (group 2), PH due to chronic lung diseases or hypoxia (group 3), chronic thromboembolic. Despite recent advances in our understanding of PH, current therapies serve only to prolong life and increase the quality of life. They are unable to reverse disease progression, and prognosis remains poor.
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