端粒 端粒酶与特发性肺间质纤维化

特发性肺纤维化(idiopathic pulmonary fibrosis, IPF) 是一种病因不明、发病机制不清、缺乏有效治疗手段的弥漫性肺间质疾病,其病理特征是肺泡上皮损伤、成纤维细胞灶(fibroblast foci) 的形成以及细胞外基质的过度积聚,最终导致了肺泡结构的异常重塑1,2].

Telomeres, telomerases and idopathic pulmonary fibrosis

Idopathic pulmonary fibrosis (IPF), with unknown pathogeny, is a interstitial lung disease.The pathological features are diffuse epithelial-cell lesion and excessive extracellular matrix deposition.Many investigators consider alveolar epithelial cells impairment and abnormal activation, fibroblast proliferation and myofibroblast differentiation as causes, resulting in excessive extracellular matrix disorganization of lung.Recently, it is believed that telomeres and telomerases might play a role in regulating the capacity of fibroblast proliferation and aberrant epithelial repair in IPF.The aim of this article is to review the telomeres, telomerases and their relationship with the idopathic pulmonary fibrosis, furthmore, to identify the etiology and pathogenesis of the idopathic pulmonary fibrosis and provide the study measure for establishing an effective treatment.