4.7 Article

Phenotypic Diversity of Vascular Smooth Muscle Cells in Pulmonary Arterial Hypertension Implications for Therapy

期刊

CHEST
卷 161, 期 1, 页码 219-231

出版社

ELSEVIER
DOI: 10.1016/j.chest.2021.08.040

关键词

contractile; synthetic phenotype; pulmonary hypertension; pulmonary vascular remodeling; smooth muscle heterogeneity; translational targets

资金

  1. Fondation Juchum
  2. Ligue Pulmonaire Vaudoise
  3. Ile de France region (ARDoc Health)

向作者/读者索取更多资源

This review discusses the origin and function of contractile cells in the pulmonary vascular wall in pulmonary arterial hypertension (PAH), as well as potential therapeutic targets and future research directions.
Pulmonary arterial hypertension (PAH) is a progressive incurable condition that is characterized by extensive remodeling of the pulmonary circulation, leading to severe right-sided heart failure and death. Similar to other vascular contractile cells, pulmonary arterial smooth muscle cells play central roles in physiological and pathologic vascular remodeling because of their remarkable ability to dynamically modulate their phenotype to ensure contractile and synthetic functions. The dysfunction and molecular mechanisms underlying their contribution to the various pulmonary vascular lesions associated with PAH have been a major focus of research. The aim of this review is to describe the medial and nonmedial origins of contractile cells in the pulmonary vascular wall and present evidence of how they contribute to the onset and progression of PAH. We also highlight specific potential target molecules and discuss future directions that are being explored to widen the therapeutic options for the treatment of PAH.

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