Pulmonary Hypertension and the Extracellular Matrix

摘要: The extracellular matrix (ECM) plays a pivotal role in regulating cell shape and signaling, maintaining cell–cell communication, controlling differentiation dedifferentiation. stiffness of vessel is directly related to the proportion various ECM components. It not surprising then that even haploinsufficiency single component such as elastin or fibrillin can lead profound developmental abnormalities propensity diseases blood vessels. For example, (Williams syndrome) associated with pulmonary systemic stenoses (Marfan leads aneurismal dilatation aorta. A variety growth factors cytokines regulate production components are fact bound by different ECM. Turnover release controlled balance between proteolytic enzymes serine elastases metalloproteinases (MMPs) their endogenous inhibitors. So it regulation would contribute fundamental way pathobiology vascular disease leading arterial hypertension (PAH).