Department of Medicine, University of Cambridge School of Clinical Medicine, Cambridge, United Kingdom.
Compr Physiol. 2011 Jan;1(1):295-317. doi: 10.1002/cphy.c100026.
Pulmonary hypertension is a multifactorial disease characterized by sustained elevation of pulmonary vascular resistance (PVR) and pulmonary arterial pressure (PAP). Central to the pathobiology of this disease is the process of vascular remodelling. This process involves structural and functional changes to the normal architecture of the walls of pulmonary arteries (PAs) that lead to increased muscularization of the muscular PAs, muscularization of the peripheral, previously nonmuscular, arteries of the respiratory acinus, formation of neointima, and formation of plexiform lesions. Underlying or contributing to the development of these lesions is hypertrophy, proliferation, migration, and resistance to apoptosis of medial cells and this article is concerned with the cellular and molecular mechanisms of these processes. In the first part of the article we focus on the concept of smooth muscle cell phenotype and the difficulties surrounding the identification and characterization of the cell/cells involved in the remodelling of the vessel media and we review the general mechanisms of cell hypertrophy, proliferation, migration and apoptosis. Then, in the larger part of the article, we review the factors identified thus far to be involved in PH intiation and/or progression and review and discuss their effects on pulmonary artery smooth muscle cells (PASMCs) the predominant cells in the tunica media of PAs.
肺动脉高压是一种多因素疾病，其特征为肺血管阻力（PVR）和肺动脉压力（PAP）持续升高。这种疾病的病理生物学的核心是血管重构过程。该过程涉及肺血管（PAs）正常壁的结构和功能变化，导致肌型 PAs 肌肉化、呼吸小叶先前非肌型的外围动脉肌肉化、新生内膜形成和丛状病变形成。这些病变的发生或促成与中膜细胞的肥大、增殖、迁移和抗凋亡有关，本文关注这些过程的细胞和分子机制。在文章的第一部分，我们重点介绍平滑肌细胞表型的概念以及鉴定和描述参与血管中膜重构的细胞/细胞所面临的困难，同时回顾细胞肥大、增殖、迁移和凋亡的一般机制。然后，在文章的较大部分，我们回顾迄今为止确定的参与 PH 起始和/或进展的因素，并回顾和讨论它们对肺动脉平滑肌细胞（PASMCs）的影响，PASMCs 是 PAs 中膜的主要细胞。
