However dysfunctional endothelial cells (ECs) could, by endothelial-to-mesenchymal transition (EndMT) 4, 5 contribute to neointima formation either directly, or indirectly by transforming in a way that impedes their ability to produce factors such as apelin that control smooth muscle cell (SMC) proliferation 6.ĮndMT is a process by which endothelial cells acquire a mesenchymal phenotype in association with expression of SMC genes, such as αSMA 4 and phospho(p) vimentin, and reduction in endothelial genes such as VE-cadherin and PECAM-1 (CD144 and CD31, respectively). The origin of the neointimal cells remains unclear they were originally thought to be derived from the muscular media as they express alpha smooth muscle actin (αSMA) 3. Abnormal muscularization and loss of pre-capillary PAs is followed by proliferation of vascular cells in more proximal PAs to form an occlusive neointima 2.
Pulmonary arterial hypertension (PAH), whether idiopathic (IPAH), heritable (HPAH) or associated with other conditions (APAH) is a potentially lethal disease characterized by progressive vascular changes leading to obliteration of distal pulmonary arteries (PAs) 1.
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