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.

弹性蛋白是一种长寿命的纤维蛋白，在肺细胞外基质中含量丰富。弹性纤维为肺部提供吸气时的特有弹性和呼气时的反冲力，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的过度沉积会损害通气和损害气体交换，从而降低肺顺应性。值得注意的是，弹性组织变性的程度与间质性肺疾病患者的肺功能和生存率的逐渐下降有关。目前，还没有经过验证的疗法可以有效减轻肺部弹性蛋白负担，也不能预防弹性组织失调。这篇综述描述了弹性蛋白在健康肺部中的作用，总结了肺部疾病中的弹性蛋白变性，并评估了目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，以开发新颖有效的疗法。