The Fra-2 transgenic mouse model of systemic sclerosis
The Fra-2 transgenic mouse model of systemic sclerosis
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DOI：
10.1016/j.vph.2012.12.001
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发表时间：
2013-03-01
影响因子：
4
通讯作者：
Distler, Oliver
中科院分区：
文献类型：
--
作者：
Maurer, Britta;Distler, Joerg H. W.;Distler, Oliver
在系统性硬化症中，微血管损伤通常先于纤维化的发展。虽然指溃疡和皮肤纤维化的发展导致高发病率，但内脏器官的影响，特别是间质性肺病和肺（动脉）高血压等并发症，导致这些患者的高疾病相关死亡率。系统性硬化症的血管动物模型对于研究病理生理学方面、识别分子关键参与者以及进行概念研究的干预性证明至关重要。迄今为止，系统性硬化症的动物模型主要反映了人类疾病的促纤维化特征。Fra-2（Fos相关抗原-2）转基因小鼠模型同时显示人类系统性硬化症的促纤维化和血管特征。(C)2012 Elsevier Inc. All rights reserved.
In systemic sclerosis, microvascular injury often precedes the development of fibrosis. Whereas the development of digital ulcers and skin fibrosis causes high morbidity, the affection of internal organs, in particular complications such as interstitial lung disease and pulmonary (arterial) hypertension, account for the high disease-associated mortality of these patients. Vascular animal models of systemic sclerosis are of utmost importance to study pathophysiological aspects, to identify molecular key players, and to perform interventional proof of concept-studies. So far, animal models of systemic sclerosis have mainly reflected the pro-fibrotic features of the human disease. The Fra-2 (Fos-related antigen-2) transgenic mouse model simultaneously displays both pro-fibrotic and vascular characteristics of human systemic sclerosis. (C) 2012 Elsevier Inc. All rights reserved.