Niiya M, Uemura M, Zheng X W, Pollak E S, Dockal M, Scheiflinger F, Wells R G, Zheng X L
Department of Pathology and Laboratory Medicine, The Children's Hospital of Philadelphia, 34th Street & Civic Center Boulevard, Philadelphia, PA 19104, USA.
J Thromb Haemost. 2006 May;4(5):1063-70. doi: 10.1111/j.1538-7836.2006.01893.x.
ADAMTS-13 is a member of A Disintegrin And Metalloprotease with ThromboSpondin type 1 repeats (ADAMTS) family, primarily synthesized in hepatic stellate cells (HSCs), one of the major cell types transdifferentiating into myofibroblasts during liver fibrosis. However, the association between ADAMTS-13 expression and HSC activation or liver fibrosis is not known.
In this study, we determined the ADAMTS-13 mRNA, protein, and activity in isolated primary HSCs upon activation on a plastic dish and in liver after administration of carbon tetrachloride (CCl(4)) in rats.
We showed that ADAMTS-13 antigen and proteolytic activity in the activated rat HSCs were dramatically increased, whereas ADAMTS-13 mRNA in these cells was only minimally altered. Similarly, the ADAMTS-13 antigen and proteolytic activity in rat liver after CCl(4) injury were also significantly increased, whereas the ADAMTS-13 mRNAs in these liver tissues were only slightly increased compared with normal. Surprisingly, despite the dramatic up-regulation of ADAMTS-13 protein synthesis in the activated HSCs after CCl(4) administration, the plasma levels of ADAMTS-13 protease in rats did not increase concordantly.
We conclude that the up-regulation of ADAMTS-13 protein expression in rat HSCs during activation in vitro and in vivo suggests the possibility of ADAMTS-13 proteolysis, an important part of function of the activated HSCs, perhaps through modulation of liver regeneration or formation of liver fibrosis after various injuries. The data also suggest the minimal contribution of the activated HSCs in regulation of plasma levels of ADAMTS-13 protease.
ADAMTS-13是具有I型血小板反应蛋白基序的去整合素和金属蛋白酶(ADAMTS)家族的成员之一,主要在肝星状细胞(HSCs)中合成,肝星状细胞是肝纤维化过程中向肌成纤维细胞转分化的主要细胞类型之一。然而,ADAMTS-13表达与肝星状细胞激活或肝纤维化之间的关联尚不清楚。
在本研究中,我们测定了在塑料培养皿上激活的原代分离肝星状细胞以及给予大鼠四氯化碳(CCl₄)后肝脏中的ADAMTS-13 mRNA、蛋白和活性。
我们发现,激活的大鼠肝星状细胞中的ADAMTS-13抗原和蛋白水解活性显著增加,而这些细胞中的ADAMTS-13 mRNA仅有微小改变。同样,CCl₄损伤后大鼠肝脏中的ADAMTS-13抗原和蛋白水解活性也显著增加,而与正常肝脏相比,这些肝脏组织中的ADAMTS-13 mRNA仅略有增加。令人惊讶的是,尽管给予CCl₄后激活的肝星状细胞中ADAMTS-13蛋白合成显著上调,但大鼠血浆中ADAMTS-13蛋白酶水平并未相应增加。
我们得出结论,大鼠肝星状细胞在体外和体内激活过程中ADAMTS-13蛋白表达上调表明存在ADAMTS-13蛋白水解的可能性,这是激活的肝星状细胞功能的重要组成部分,可能是通过调节各种损伤后的肝再生或肝纤维化形成来实现的。数据还表明激活的肝星状细胞对ADAMTS-13蛋白酶血浆水平的调节作用极小。