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骨膜蛋白在肺纤维化和气道重塑中的作用。

The role of periostin in lung fibrosis and airway remodeling.

作者信息

O'Dwyer David N, Moore Bethany B

机构信息

Department of Internal Medicine, Division of Pulmonary and Critical Care Medicine, University of Michigan, 4053 BSRB, 109 Zina Pitcher Place, Ann Arbor, MI, 48109-2200, USA.

Department of Microbiology and Immunology, University of Michigan, Ann Arbor, MI, USA.

出版信息

Cell Mol Life Sci. 2017 Dec;74(23):4305-4314. doi: 10.1007/s00018-017-2649-z. Epub 2017 Sep 16.

Abstract

Periostin is a protein that plays a key role in development and repair within the biological matrix of the lung. As a matricellular protein that does not contribute to extracellular matrix structure, periostin interacts with other extracellular matrix proteins to regulate the composition of the matrix in the lung and other organs. In this review, we discuss the studies exploring the role of periostin to date in chronic respiratory diseases, namely asthma and idiopathic pulmonary fibrosis. Asthma is a major health problem globally affecting millions of people worldwide with significant associated morbidity and mortality. Periostin is highly expressed in the lungs of asthmatic patients, contributes to mucus secretion, airway fibrosis and remodeling and is recognized as a biomarker of Th2 high inflammation. Idiopathic pulmonary fibrosis is a fatal interstitial lung disease characterized by progressive aberrant fibrosis of the lung matrix and respiratory failure. It predominantly affects adults over 50 years of age and its incidence is increasing worldwide. Periostin is also highly expressed in the lungs of idiopathic pulmonary fibrosis patients. Serum levels of periostin may predict clinical progression in this disease and periostin promotes myofibroblast differentiation and type 1 collagen production to contribute to aberrant lung fibrosis. Studies to date suggest that periostin is a key player in several pathogenic mechanisms within the lung and may provide us with a useful biomarker of clinical progression in both asthma and idiopathic pulmonary fibrosis.

摘要

骨膜蛋白是一种在肺生物基质的发育和修复中起关键作用的蛋白质。作为一种不构成细胞外基质结构的基质细胞蛋白,骨膜蛋白与其他细胞外基质蛋白相互作用,以调节肺和其他器官中基质的组成。在本综述中,我们讨论了迄今为止探索骨膜蛋白在慢性呼吸道疾病(即哮喘和特发性肺纤维化)中作用的研究。哮喘是一个全球性的主要健康问题,影响着全球数百万人,伴有显著的发病率和死亡率。骨膜蛋白在哮喘患者的肺中高度表达,促进黏液分泌、气道纤维化和重塑,并被认为是Th2高炎症的生物标志物。特发性肺纤维化是一种致命的间质性肺疾病,其特征是肺基质进行性异常纤维化和呼吸衰竭。它主要影响50岁以上的成年人,并且在全球范围内其发病率正在上升。骨膜蛋白在特发性肺纤维化患者的肺中也高度表达。骨膜蛋白的血清水平可能预测该疾病的临床进展,并且骨膜蛋白促进肌成纤维细胞分化和I型胶原蛋白产生,从而导致异常的肺纤维化。迄今为止的研究表明,骨膜蛋白是肺内几种致病机制中的关键因素,并且可能为我们提供哮喘和特发性肺纤维化临床进展的有用生物标志物。

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