Developmental pathways in the pathogenesis of lung fibrosis.
Developmental pathways in the pathogenesis of lung fibrosis.
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DOI：
10.1016/j.mam.2018.08.004
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发表时间：
2019-03
影响因子：
10.6
通讯作者：
Thannickal VJ
中科院分区：
文献类型：
--
作者：
Chanda D;Otoupalova E;Smith SR;Volckaert T;De Langhe SP;Thannickal VJ
特发性肺纤维化（IPF）是一种进行性和终末期肺部疾病，目前尚无治愈方法。IPF是一种衰老性疾病，诊断的中位年龄超过65岁。诊断后的中位生存期为3-5年。IPF的主要特征是活化的肺成纤维细胞和肌成纤维细胞过度沉积细胞外基质（ECM）蛋白，导致气体交换减少和肺功能受损。越来越多的证据支持由潜在因素如遗传易感性、慢性损伤和衰老、氧化应激和受损的再生反应精心策划的促纤维化环境的概念，这些因素可能导致疾病的发展和持续。目前，两种FDA批准的药物在治疗IPF方面的疗效有限。许多与肺发育相关的基因和基因网络在IPF中被诱导或激活。在这篇综述中，我们分析了该领域的现有知识，从基础和临床研究中获得的，提供新的见解的疾病过程，和潜在的方法来治疗肺纤维化。
Idiopathic pulmonary fibrosis (IPF) is a progressive and terminal lung disease with no known cure. IPF is a disease of aging, with median age of diagnosis over 65 years. Median survival is between 3–5 years after diagnosis. IPF is characterized primarily by excessive deposition of extracellular matrix (ECM) proteins by activated lung fibroblasts and myofibroblasts, resulting in reduced gas exchange and impaired pulmonary function. Growing evidence supports the concept of a pro-fibrotic environment orchestrated by underlying factors such as genetic predisposition, chronic injury and aging, oxidative stress, and impaired regenerative responses may account for disease development and persistence. Currently, two FDA approved drugs have limited efficacy in the treatment of IPF. Many of the genes and gene networks associated with lung development are induced or activated in IPF. In this review, we analyze current knowledge in the field, gained from both basic and clinical research, to provide new insights into the disease process, and potential approaches to treatment of pulmonary fibrosis.
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影响因子：
6
作者：
Ahlfeld, Shawn K.;Wang, Jian;Conway, Simon J.
通讯作者：
Conway, Simon J.
影响因子：
16.6
作者：
Alanis, Denise Martinez;Chang, Daniel R.;Akiyama, Haruhiko;Krasnow, Mark A.;Chen, Jichao
通讯作者：
Chen, Jichao
Notch 和 TGF-β 信号通路之间的串扰由 Notch 胞内结构域与 Smad3 的相互作用介导。
影响因子：
7.8
作者：
Blokzijl, A;Dahlqvist, C;Reissmann, E;Falk, A;Moliner, A;Lendahl, U;Ibáñez, CF
通讯作者：
Ibáñez, CF
DOI：
10.1038/nrd2792
发表时间：
2009-03
期刊：
Nature reviews. Drug discovery
影响因子：
--
作者：
通讯作者：
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影响因子：
4.8
作者：
Bhaskaran, Manoj;Kolliputi, Narasaiah;Liu, Lin
通讯作者：
Liu, Lin