The Role of PPAR Gamma in Systemic Sclerosis.

The Role of PPAR Gamma in Systemic Sclerosis.
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DOI:
10.1155/2015/124624
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发表时间:
2015
期刊:
影响因子:
2.9
通讯作者:
Pitta MG
Pitta MG
中科院分区:
医学3区
文献类型:
--
作者:
Dantas AT;Pereira MC;de Melo Rego MJ;da Rocha LF Jr;Pitta Ida R;Marques CD;Duarte AL;Pitta MG

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纤维化被认为是许多慢性疾病的重要特征,如系统性硬化症(SSC),这是一种病因不明的自身免疫性疾病,以免疫调节失调和血管损伤为特征,随后累及皮肤和多个内脏器官的进行性纤维化。SSc的预后很差,因为目前还没有显示出逆转或阻止纤维化进展的治疗方法,这是一个主要的未得到满足的医学需求。近年来,PPARγ配体的抗纤维化作用已在体内外进行了研究,并出现了一些新的理论和见解。PPARγ功能异常似乎与皮肤和肺的病理性纤维化有关。其抗纤维化作用主要与抑制转化生长因子-β/Smad信号转导有关,但也可通过其他途径发挥作用。本文综述了近年来的研究,发现PPARγ是一个重要的新途径,在调节结缔组织动态平衡方面具有重要作用,重点是皮肤和肺纤维化及其在系统性硬化症中的作用。
Fibrosis is recognized as an important feature of many chronic diseases, such as systemic sclerosis (SSc), an autoimmune disease of unknown etiology, characterized by immune dysregulation and vascular injury, followed by progressive fibrosis affecting the skin and multiple internal organs. SSc has a poor prognosis because no therapy has been shown to reverse or arrest the progression of fibrosis, representing a major unmet medical need. Recently, antifibrotic effects of PPARγ ligands have been studied in vitro and in vivo and some theories have emerged leading to new insights. Aberrant PPARγ function seems to be implicated in pathological fibrosis in the skin and lungs. This antifibrotic effect is mainly related to the inhibition of TGF-β/Smad signal transduction but other pathways can be involved. This review focused on recent studies that identified PPARγ as an important novel pathway with critical roles in regulating connective tissue homeostasis, with emphasis on skin and lung fibrosis and its role on systemic sclerosis.
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