Shi Zengdun, Rockey Don C
Department of medicine, Medical University of South Carolina, Charleston, SC, USA.
Lab Invest. 2017 Dec;97(12):1412-1426. doi: 10.1038/labinvest.2017.96. Epub 2017 Oct 16.
Liver fibrosis, a model wound healing system, is characterized by excessive deposition of extracellular matrix (ECM) in the liver. Although many fibrogenic cell types may express ECM, the hepatic stellate cell (HSC) is currently considered to be the major effector. HSCs transform into myofibroblast-like cells, also known as hepatic myofibroblasts in a process known as activation; this process is characterized in particular by de novo expression of smooth muscle alpha actin (SM α-actin) and type 1 collagen. The family of actins, which form the cell's cytoskeleton, are essential in many cellular processes. β-actin and cytoplasmic γ-actin (γ-actin) are ubiquitously expressed, whereas SM α-actin defines smooth muscle cell and myofibroblast phenotypes. Thus, SM α-actin is tightly associated with multiple functional properties. However, the regulatory mechanisms by which actin isoforms might regulate type 1 collagen remain unclear. In primary HSCs from normal and fibrotic rat liver, we demonstrate that myocardin, a canonical SRF cofactor, is upregulated in hepatic myofibroblasts and differentially regulates SM α-actin, γ-actin, and β-actins through activation of an ATTA box in the SM α-actin and a CCAAT box in γ-actin and β-actin promoters, respectively; moreover, myocardin differentially activated serum response factor (SRF) in CArG boxes of actin promoters. In addition, myocardin-stimulated Smad2 phosphorylation and RhoA expression, leading to increased expression of type 1 collagen in an actin cytoskeleton-dependent manner. Myocardin also directly enhanced SRF expression and stimulated collagen 1α1 and 1α2 promoter activities. In addition, overexpression of myocardin in vivo during carbon tetrachloride-induced liver injury led to increased HSC activation and fibrogenesis. In summary, our data suggest that myocardin plays a critical role in actin cytoskeletal dynamics during HSC activation, in turn, specifically regulating type I collagen expression in hepatic myofibroblasts.
肝纤维化是一种典型的伤口愈合系统,其特征是肝脏中细胞外基质(ECM)过度沉积。尽管许多促纤维化细胞类型都可能表达ECM,但肝星状细胞(HSC)目前被认为是主要效应细胞。HSC在一个称为激活的过程中转化为肌成纤维细胞样细胞,也称为肝肌成纤维细胞;这个过程的特点尤其在于平滑肌α肌动蛋白(SMα-肌动蛋白)和I型胶原蛋白的从头表达。肌动蛋白家族构成细胞的细胞骨架,在许多细胞过程中至关重要。β-肌动蛋白和细胞质γ-肌动蛋白(γ-肌动蛋白)广泛表达,而SMα-肌动蛋白则定义了平滑肌细胞和肌成纤维细胞的表型。因此,SMα-肌动蛋白与多种功能特性密切相关。然而,肌动蛋白异构体调节I型胶原蛋白的机制仍不清楚。在来自正常和纤维化大鼠肝脏的原代HSC中,我们证明,作为典型的血清反应因子(SRF)辅因子的心肌蛋白在肝肌成纤维细胞中上调,并分别通过激活SMα-肌动蛋白启动子中的ATTA盒以及γ-肌动蛋白和β-肌动蛋白启动子中的CCAAT盒来差异调节SMα-肌动蛋白、γ-肌动蛋白和β-肌动蛋白;此外,心肌蛋白在肌动蛋白启动子的CArG盒中差异激活血清反应因子(SRF)。此外,心肌蛋白刺激Smad2磷酸化和RhoA表达,导致I型胶原蛋白以肌动蛋白细胞骨架依赖的方式表达增加。心肌蛋白还直接增强SRF表达并刺激胶原蛋白1α1和1α2启动子活性。此外,在四氯化碳诱导的肝损伤期间在体内过表达心肌蛋白导致HSC激活和纤维化增加。总之,我们的数据表明,心肌蛋白在HSC激活过程中的肌动蛋白细胞骨架动态中起关键作用,进而特异性调节肝肌成纤维细胞中I型胶原蛋白的表达。