Hazard Identification Division, Environmental Health Science and Research Bureau, Health Canada, Ottawa, Ontario, Canada.
Am J Pathol. 2012 Apr;180(4):1413-30. doi: 10.1016/j.ajpath.2011.12.020. Epub 2012 Feb 7.
Increased production of tumor necrosis factor (TNF)-α and matrix metalloproteinases (MMPs) is a feature of inflammatory lung diseases, including emphysema and fibrosis, but the divergent pathological characteristics that result indicate involvement of other processes in disease pathogenesis. Transgenic mice overexpressing TNF-α in type II alveolar epithelial cells under the control of the surfactant protein (SP)-C promoter develop pulmonary inflammation and emphysema but are resistant to induction of fibrosis by administration of bleomycin or transforming growth factor-β. To study the molecular mechanisms underlying the development of this phenotype, we used a microarray approach to characterize the pulmonary transcriptome of SP-C/TNF-α mice and wild-type littermates. Four-month-old SP-C/TNF-α mice displayed pronounced pulmonary inflammation, airspace enlargement, increased MMP-2 and MMP-9 levels, and altered expression of 2332 probes. The functional assessment of genes with increased expression revealed enrichment of inflammatory/immune responses and proteases, whereas genes involved in protease inhibition, angiogenesis, cross-linking of basement membrane proteins, and myofibroblast differentiation were predominantly decreased. Comparison with multiple lung disease models identified a set of genes unique to the SP-C/TNF-α model and revealed that lack of extracellular matrix production distinguished SP-C/TNF-α mice from fibrosis models. Activation of inflammatory and proteolytic pathways and disruption of maintenance and repair processes are central features of emphysema in this TNF-overexpression model. Impairment of myofibroblast differentiation and extracellular matrix production may underlie resistance to induction of fibrosis.
肿瘤坏死因子 (TNF)-α 和基质金属蛋白酶 (MMPs) 的产量增加是炎症性肺疾病(包括肺气肿和纤维化)的特征,但导致的不同病理特征表明,其他过程也参与了疾病的发病机制。在表面活性蛋白 (SP)-C 启动子的控制下,在 II 型肺泡上皮细胞中过表达 TNF-α 的转基因小鼠会发生肺部炎症和肺气肿,但对博莱霉素或转化生长因子-β诱导的纤维化有抗性。为了研究这种表型发展的分子机制,我们使用微阵列方法来描述 SP-C/TNF-α 小鼠和野生型同窝仔鼠的肺转录组。四个月大的 SP-C/TNF-α 小鼠表现出明显的肺部炎症、气腔扩大、MMP-2 和 MMP-9 水平升高,以及 2332 个探针的表达改变。对表达增加的基因的功能评估显示,炎症/免疫反应和蛋白酶的富集,而参与蛋白酶抑制、血管生成、基底膜蛋白交联和肌成纤维细胞分化的基因则主要减少。与多种肺部疾病模型的比较确定了一组仅存在于 SP-C/TNF-α 模型中的基因,并表明缺乏细胞外基质产生将 SP-C/TNF-α 小鼠与纤维化模型区分开来。炎症和蛋白水解途径的激活以及维持和修复过程的破坏是该 TNF 过表达模型中肺气肿的主要特征。肌成纤维细胞分化和细胞外基质产生的受损可能是对纤维化诱导产生抗性的基础。