Altered Purinergic Signalling in Endothelial Cells ... - Helda

Altered Purinergic Signalling in Endothelial Cells ... - Helda

media, due to SMC proliferation and migration (Heath and Edwards, 1958). Other

associated features are increased EC injury and apoptosis, which leads to loss of precapillary

arteries and thus leads to critical changes in pulmonary hemodynamics (Jurasz

et al, 2010). During the course of PAH, endothelial cells with hyper-proliferative and

apoptosis-resistant phenotype appear and contribute to intima hyperplasia and occlusion

of arteries (Masri et al, 2007; Kawano, 1994; Magee et al, 1988). Hallmarks for endstage

severe PAH is plexogenic arteriopathy characterized by tumor-like cell mass with

capillary-like channels (Figure 2). Pathobiology of these lesions is still a matter of

debate and the origin of involved cells is also obscure (Yi et al, 2000; Abe et al, 2010;

Toshner et al, 2009).

Figure 1. Vascular layers of healthy muscular artery. Hypertrophy of intima and media

layers are hallmarks of IPAH. (240113,


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