Fibrosis in connective tissue disease: the role of the myofibroblast and fibroblast-epithelial cell interactions.

Fibrosis in connective tissue disease: the role of the myofibroblast and fibroblast-epithelial cell interactions.
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结缔组织疾病中的纤维化:肌纤维细胞和成纤维细胞上皮细胞相互作用的作用。

DOI:
10.1186/ar2188
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发表时间:
2007
影响因子:
4.9
通讯作者:
Lafyatis, Robert
Lafyatis, Robert
中科院分区:
医学2区
文献类型:
--
作者:
Krieg, Thomas;Abraham, David;Lafyatis, Robert

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以细胞外基质过度积累为特征的纤维化是许多结缔组织疾病的共同特征,尤其是硬皮病(系统性硬化症)。实验研究表明,涉及内皮细胞、上皮细胞、成纤维细胞和免疫细胞的细胞间相互作用的复杂网络使用一系列分子介质驱动导致纤维化的致病事件。转化生长因子-β和内皮素-1是结缔组织生长因子的细胞因子层级的一部分,是纤维发生的关键介质,主要负责成纤维细胞向肌成纤维细胞表型分化。皮肤纤维化的紧皮小鼠(Tsk-1)模型表明,许多其他基因也可能是重要的。
Fibrosis, characterized by excessive extracellular matrix accumulation, is a common feature of many connective tissue diseases, notably scleroderma (systemic sclerosis). Experimental studies suggest that a complex network of intercellular interactions involving endothelial cells, epithelial cells, fibroblasts and immune cells, using an array of molecular mediators, drives the pathogenic events that lead to fibrosis. Transforming growth factor-β and endothelin-1, which are part of a cytokine hierarchy with connective tissue growth factor, are key mediators of fibrogenesis and are primarily responsible for the differentiation of fibroblasts toward a myofibroblast phenotype. The tight skin mouse (Tsk-1) model of cutaneous fibrosis suggests that numerous other genes may also be important.
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影响因子: 6.7
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