Clark Stephen H
University of Connecticut Health Center, Farmington, CT 06030, USA.
Curr Rheumatol Rep. 2005 Apr;7(2):150-5. doi: 10.1007/s11926-005-0068-x.
Scleroderma or systemic sclerosis is an insidious connective tissue disease with no known cure. A hallmark feature of scleroderma is the excess synthesis and deposition of collagen resulting in a fibrotic state. In scleroderma, fibrosis is not confined only to the skin but impacts internal organs as well. In an effort to better understand the pathophysiology of this disease, researchers have developed a variety of animal models that display features of the human condition. This paper focuses on mouse models of scleroderma and summarizes work conducted with these experimental paradigms that is focused on understanding the cellular and molecular events associated with the onset and maintenance of fibrosis.
硬皮病或系统性硬化症是一种隐匿性结缔组织疾病，目前尚无治愈方法。硬皮病的一个标志性特征是胶原蛋白过度合成和沉积，导致纤维化状态。在硬皮病中，纤维化不仅局限于皮肤，还会影响内部器官。为了更好地理解这种疾病的病理生理学，研究人员开发了多种表现出人类疾病特征的动物模型。本文重点关注硬皮病的小鼠模型，并总结了利用这些实验范式开展的工作，这些工作旨在了解与纤维化的发生和维持相关的细胞和分子事件。
