Lung Fibroblasts, Aging, and Idiopathic Pulmonary Fibrosis
Lung Fibroblasts, Aging, and Idiopathic Pulmonary Fibrosis
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肺成纤维细胞、衰老和特发性肺纤维化
DOI：
10.1513/annalsats.201605-341aw
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发表时间：
2016-12-01
影响因子：
8.3
通讯作者：
Selman, Moises
中科院分区：
文献类型：
--
作者：
Pardo, Annie;Selman, Moises
特发性肺纤维化（IPF）是一种与年龄相关的、进行性和不可逆的肺部疾病，病因不明，发病机制难以捉摸，治疗方案非常有限。IPF的特征是肺泡上皮细胞的异常活化、成纤维细胞和肌成纤维细胞的积累以及细胞外基质的过量产生。衰老与这种疾病的联系尚不确定，但在IPF肺中发现了一些与衰老相关的变化，包括端粒磨损、细胞衰老和线粒体功能障碍。此外，衰老似乎赋予成纤维细胞纤维化表型，并增加非ipf纤维化性肺疾病的纤维化反应的严重程度。更好地了解将衰老与IPF联系起来的病理生理机制将促进对其发病机制的理解，并可能为治疗这种毁灭性疾病提供新的治疗窗口。
Idiopathic pulmonary fibrosis (IPF) is an aging-associated, progressive, and irreversible lung disease of unknown etiology, elusive pathogenesis, and very limited therapeutic options. The hallmarks of IPF are aberrant activation of alveolar epithelial cells and accumulation of fibroblasts and myofibroblasts along with excessive production of extracellular matrix. The linkage of aging with this disorder is uncertain, but a number of changes associated with aging, including telomere attrition, cell senescence, and mitochondrial dysfunction, have been revealed in IPF lungs. Also, aging seems to confer a profibrotic phenotype upon fibroblasts and to increase the severity of the fibrogenic response in non-IPF fibrotic lung disorders. Better knowledge of the pathophysiological mechanisms linking aging to IPF will advance understanding of its pathogenesis and may provide new therapeutic windows to treatment of this devastating disease.