Elastin in the Liver.

Elastin in the Liver.
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肝脏中的弹性。

DOI:
10.3389/fphys.2016.00491
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发表时间:
2016
影响因子:
4
通讯作者:
Kanta J
Kanta J
中科院分区:
医学2区
文献类型:
--
作者:
Kanta J

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肝硬化的一个特征是大量结缔组织的积累,其主要成分是I型胶原蛋白。弹性蛋白在正常肝脏中是一种次要的结缔组织成分,但在病变肝脏中由肝星状细胞和门静脉成纤维细胞积极合成。肝纤维化晚期弹性纤维的积累可能导致疾病的可逆性随着时间的推移而降低。弹性蛋白是由对偶弹性蛋白单体聚合形成的。它是一种无定形的蛋白质,高度抵抗形成弹性纤维核心的蛋白酶的作用。核心周围的微原纤维由纤维蛋白组成,这些纤维蛋白结合了一些参与纤维形成的蛋白质。它们包括微纤维相关糖蛋白(MAGPs)、微纤维相关蛋白(MFAPs)和纤维蛋白。赖氨酸氧化酶(LOX)和赖氨酸氧化酶样蛋白(LOXLs)负责对流层弹性蛋白交联和聚合。附着在微原纤维上的TGF-β复合物释放这种细胞因子并影响邻近细胞的行为。TGF-β在肝脏中作为主要促纤维化细胞因子的作用是众所周知的,TGF-β超家族细胞因子从其储存在弹性纤维中的释放可能影响纤维化的过程。弹性纤维经常在组织中进行研究,它们提供弹性和弹性,但它们的作用不再被视为纯粹的机械作用。部分弹性蛋白降解产生的弹力蛋白、弹性蛋白聚合物和弹性蛋白肽影响成纤维细胞和炎症细胞以及血管生成。肝脏中的弹性蛋白也起着类似的作用。本文就肝脏弹性纤维在弹性蛋白生化和生理学方面的研究进展作一综述。肝弹性蛋白合成和降解的调节可能对肝纤维化的结果很重要。
A characteristic feature of liver cirrhosis is the accumulation of large amounts of connective tissue with the prevailing content of type I collagen. Elastin is a minor connective tissue component in normal liver but it is actively synthesized by hepatic stellate cells and portal fibroblasts in diseased liver. The accumulation of elastic fibers in later stages of liver fibrosis may contribute to the decreasing reversibility of the disease with advancing time. Elastin is formed by polymerization of tropoelastin monomers. It is an amorphous protein highly resistant to the action of proteases that forms the core of elastic fibers. Microfibrils surrounding the core are composed of fibrillins that bind a number of proteins involved in fiber formation. They include microfibril-associated glycoproteins (MAGPs), microfibrillar-associated proteins (MFAPs) and fibulins. Lysyl oxidase (LOX) and lysyl oxidase-like proteins (LOXLs) are responsible for tropoelastin cross-linking and polymerization. TGF-β complexes attached to microfibrils release this cytokine and influence the behavior of the cells in the neighborhood. The role of TGF-β as the main profibrotic cytokine in the liver is well-known and the release of the cytokines of TGF-β superfamily from their storage in elastic fibers may affect the course of fibrosis. Elastic fibers are often studied in the tissues where they provide elasticity and resilience but their role is no longer viewed as purely mechanical. Tropoelastin, elastin polymer and elastin peptides resulting from partial elastin degradation influence fibroblastic and inflammatory cells as well as angiogenesis. A similar role may be performed by elastin in the liver. This article reviews the results of the research of liver elastic fibers on the background of the present knowledge of elastin biochemistry and physiology. The regulation of liver elastin synthesis and degradation may be important for the outcome of liver fibrosis.
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