Journal
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
Volume 11, Issue -, Pages -Publisher
FRONTIERS MEDIA SA
DOI: 10.3389/fcell.2023.1132060
Keywords
pulmonary hypertension; pulmonary vascular remodeling; transcription factors; endothelial cells; pulmonary arterial smooth muscle cells
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Pulmonary hypertension is a fatal disease with excessive pulmonary arterial cell remodeling. Various cellular abnormalities contribute to pulmonary arterial remodeling, leading to increased pulmonary vascular resistance and pressure. Studies have identified transcription factors as key regulators in this process, highlighting the importance of understanding their molecular mechanisms for developing novel therapies.
Pulmonary hypertension (PH) is a refractory and fatal disease characterized by excessive pulmonary arterial cell remodeling. Uncontrolled proliferation and hypertrophy of pulmonary arterial smooth muscle cells (PASMCs), dysfunction of pulmonary arterial endothelial cells (PAECs), and abnormal perivascular infiltration of immune cells result in pulmonary arterial remodeling, followed by increased pulmonary vascular resistance and pulmonary pressure. Although various drugs targeting nitric oxide, endothelin-1 and prostacyclin pathways have been used in clinical settings, the mortality of pulmonary hypertension remains high. Multiple molecular abnormalities have been implicated in pulmonary hypertension, changes in numerous transcription factors have been identified as key regulators in pulmonary hypertension, and a role for pulmonary vascular remodeling has been highlighted. This review consolidates evidence linking transcription factors and their molecular mechanisms, from pulmonary vascular intima PAECs, vascular media PASMCs, and pulmonary arterial adventitia fibroblasts to pulmonary inflammatory cells. These findings will improve the understanding of particularly interactions between transcription factor-mediated cellular signaling pathways and identify novel therapies for pulmonary hypertension.
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