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The Roles of Tenascins in Cardiovascular, Inflammatory, and Heritable Connective Tissue Diseases
Frontiers in Immunology ( IF 5.7 ) Pub Date : 2020-11-03 , DOI: 10.3389/fimmu.2020.609752
Ken-Ichi Matsumoto 1 , Hiroki Aoki 2
Affiliation  

Tenascins are a family of multifunctional extracellular matrix (ECM) glycoproteins with time- and tissue specific expression patterns during development, tissue homeostasis, and diseases. There are four family members (tenascin-C, -R, -X, -W) in vertebrates. Among them, tenascin-X (TNX) and tenascin-C (TNC) play important roles in human pathologies. TNX is expressed widely in loose connective tissues. TNX contributes to the stability and maintenance of the collagen network, and its absence causes classical-like Ehlers-Danlos syndrome (clEDS), a heritable connective tissue disorder. In contrast, TNC is specifically and transiently expressed upon pathological conditions such as inflammation, fibrosis, and cancer. There is growing evidence that TNC is involved in inflammatory processes with proinflammatory or anti-inflammatory activity in a context-dependent manner. In this review, we summarize the roles of these two tenascins, TNX and TNC, in cardiovascular and inflammatory diseases and in clEDS, and we discuss the functional consequences of the expression of these tenascins for tissue homeostasis.



中文翻译:

肌腱蛋白在心血管,炎性和遗传性结缔组织疾病中的作用

肌腱蛋白是多功能细胞外基质(ECM)糖蛋白家族,在发育,组织稳态和疾病过程中具有时间和组织特异性表达模式。脊椎动物中有四个家族成员(腱生蛋白-C,-R,-X,-W)。其中,腱生蛋白X(TNX)和腱生蛋白C(TNC)在人类病理中起重要作用。TNX在松散的结缔组织中广泛表达。TNX有助于胶原蛋白网络的稳定性和维持,而其缺乏会导致经典的Ehlers-Danlos综合征(clEDS),这是一种遗传性结缔组织疾病。相反,TNC在诸如炎症,纤维化和癌症等病理状况下特异性且短暂地表达。越来越多的证据表明,TNC以上下文相关的方式参与具有促炎或抗炎活性的炎症过程。在这篇综述中,我们总结了这两种肌腱蛋白TNX和TNC在心血管疾病和炎性疾病以及clEDS中的作用,并讨论了这两种肌腱蛋白表达对组织稳态的功能影响。

更新日期:2020-12-01
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