Molecular pathogenesis of skin fibrosis: insight from animal models.
Molecular pathogenesis of skin fibrosis: insight from animal models.
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DOI：
10.1007/s11926-009-0080-7
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发表时间：
2010-02
影响因子：
5
通讯作者：
Chan, Edwin S L
中科院分区：
文献类型：
--
作者：
Smith, Gideon P;Chan, Edwin S L
皮肤纤维化发生在多种人类疾病中，最明显的是系统性硬化症（SSc）。人类皮肤硬皮病的终末阶段包括真皮中过多的胶原沉积以及附件结构和相关脂肪组织的损失。这一过程和早期阶段的启动因素被认为是通过血管损伤和免疫功能障碍以及炎症反应失调而发生的。然而，由于该病的发病隐匿，这一阶段很少在人类中观察到，并且仍然知之甚少。动物模型提供了一种方法来检查这些早期阶段，并分离和了解信号通路、趋化因子和细胞因子扰动的影响。本文综述了不同动物模型对SSc皮肤纤维化分子发病机制的最新研究进展，包括其起始和维持阶段。
Skin fibrosis occurs in a variety of human diseases, most notably systemic sclerosis (SSc). The end stage of scleroderma in human skin consists of excess collagen deposition in the dermis with loss of adnexal structures and associated adipose tissue. The initiating factors for this process and the early stages are believed to occur through vascular injury and immune dysfunction with a dysregulated inflammatory response. However, because of the insidious onset of the disease, this stage is rarely observed in humans and remains poorly understood. Animal models have provided a means to examine these early stages and to isolate and understand the effect of perturbations in signaling pathways, chemokines, and cytokines. This article summarizes recent progress in the understanding of the molecular pathogenesis of skin fibrosis in SSc from different animal models, both its initiation and its maintenance phases.