Abstract
Pulmonary arterial hypertension (PAH) is characterized by an important occlusive vascular remodeling with the production of new endothelial cells, smooth muscle cells, myofibroblasts, and fibroblasts. Identifying the cellular processes leading to vascular proliferation and dysfunction is a major goal in order to decipher the mechanisms leading to PAH development. In addition to in situ proliferation of vascular cells, studies from the past 20 years have unveiled the role of circulating and resident vascular in pulmonary vascular remodeling. This review aims at summarizing the current knowledge on the different progenitor and stem cells that have been shown to participate in pulmonary vascular lesions and on the pathways regulating their recruitment during PAH. Finally, this review also addresses the therapeutic potential of circulating endothelial progenitor cells and mesenchymal stem cells.
Highlights
The origin of the vascular remodeling occurring during the various forms of pulmonary arterial hypertension (PAH) is still unexplained, some pathological conditions or gene defects are known to favor the development of the disease
This remodeling can predominate in the arterial compartment in PAH or the venous side in pulmonary veno-occlusive disease (PVOD) through the production of new endothelial cells (EC), myofibroblasts, vascular smooth muscle cells (SMC), and through extracellular matrix changes with intimal and medial fibrosis in the intima [1]
Tsshisorfemnoodrmelinagllyinvmoluvessctuhleaprriozdeudctiaornteorf inoelwesenadnod- muscularization of previously non-muscularized arterioles. This remodeling involves the production of new endothelial cells, myofibroblasts (MF), vascular smooth muscle cells, fibroblasts, and extracellular matrix changes leading to the formation of a neointima between the endothelium and the internal elastic lamina, to medial hypertrophy and vascular/perivascular fibrosis and inflammation
Summary
The origin of the vascular remodeling occurring during the various forms of pulmonary arterial hypertension (PAH) is still unexplained, some pathological conditions or gene defects are known to favor the development of the disease. Tsshisorfemnoodrmelinagllyinvmoluvessctuhleaprriozdeudctiaornteorf inoelwesenadnod- muscularization of previously non-muscularized arterioles This remodeling involves the production of new endothelial cells, myofibroblasts (MF), vascular smooth muscle cells, fibroblasts, and extracellular matrix changes leading to the formation of a neointima between the endothelium and the internal elastic lamina, to medial hypertrophy and vascular/perivascular fibrosis and inflammation. Circulating (EPC and MSC) and resident vascular stem/progenitor cells (EPC, activated SMC, SPC, pericytes, MSC) were identified These cells can be either mobilized from cellular niches within or close to the vessel wall or in the lung interstitium, or from distant tissues, mainly the bone marrow, through the circulation. We will review the various types of progenitor and stem cells that have been shown to be involved in pulmonary vascular remodeling during pulmonary hypertension (PH) and the signaling pathways that can modulate their recruitment
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