PDF(Pubmed)
Abstract：
Elastin is a long-lived fibrous protein that is abundant in the extracellular matrix of the lung. Elastic fibers provide the lung the characteristic elasticity during inhalation with recoil during exhalation thereby ensuring efficient gas exchange. Excessive deposition of elastin and other extracellular matrix proteins reduces lung compliance by impairing ventilation and compromising gas exchange. Notably, the degree of elastosis is associated with the progressive decline in lung function and survival in patients with interstitial lung diseases. Currently there are no proven therapies which effectively reduce the elastin burden in the lung nor prevent dysregulated elastosis. This review describes elastin\'s role in the healthy lung, summarizes elastosis in pulmonary diseases, and evaluates the current understanding of elastin regulation and dysregulation with the goal of guiding future research efforts to develop novel and effective therapies.
摘要：
弹性蛋白是一种长寿命的纤维蛋白，在肺的细胞外基质中含量丰富。弹性纤维在吸气期间为肺提供特征弹性,在呼气期间具有反冲,从而确保有效的气体交换。弹性蛋白和其他细胞外基质蛋白质的过度沉积通过损害通气和损害气体交换来降低肺顺应性。值得注意的是,在间质性肺病患者中,弹性弹性增生程度与肺功能和生存率的进行性下降有关.目前,没有有效降低肺中的弹性蛋白负荷或防止异常调节的弹性沉着症的经证实的疗法。这篇综述描述了弹性蛋白在健康肺部的作用，总结了肺部疾病中的弹性沉着症，并评估当前对弹性蛋白调节和失调的理解，目的是指导未来的研究工作以开发新的有效疗法。
