Bennett Robert G
Research Service, VA Nebraska-Western Iowa Health Care System; Departments of Internal Medicine and Biochemistry & Molecular Biology, University of Nebraska Medical Center, Omaha, Nebraska, USA.
Eur Med J Hepatol. 2018 May;6(1):80-87. Epub 2018 May 31.
Hepatic fibrosis is a progressive disease with few treatment options outside of transplantation. Relaxin is a member of the insulin/relaxin superfamily of peptide hormones. Originally known for its roles in pregnancy, relaxin promotes reproductive tissue remodelling and regulates vascular changes, including increased arterial compliance and reduced vascular resistance. Outside of pregnancy, relaxin plays a major role in the protection of organs from excess extracellular matrix accumulation, as demonstrated by the relaxin-null mouse, which develops widespread fibrosis with ageing. Relaxin reduces scarring due to excess collagen deposition by inhibiting collagen production while simultaneously promoting its degradation and can reduce established fibrosis in several animal models of extracellular matrix-associated disease, including liver fibrosis. Treatment with relaxin reduces the myofibroblastic phenotype of activated hepatic stellate cells, the major hepatic collagen-producing cell in fibrosis and cirrhosis. Relaxin also has haemodynamic effects, including vasodilation, and can reduce portal hypertension associated with cirrhosis. In this review, a brief overview of hepatic fibrosis and the role of the hepatic stellate cell will be presented, followed by an introduction to relaxin and its actions. The use of relaxin to treat preclinical models of fibrotic diseases, including liver diseases, will also be discussed. Finally, the completed, current, and ongoing clinical trials of relaxin in human disease will be described, followed by the limitations and future directions for the use of relaxin for disease treatment.
肝纤维化是一种进展性疾病,除了肝移植外几乎没有其他治疗选择。松弛素是胰岛素/松弛素肽类激素超家族的成员。松弛素最初因其在妊娠中的作用而为人所知,它能促进生殖组织重塑并调节血管变化,包括增加动脉顺应性和降低血管阻力。在非妊娠状态下,松弛素在保护器官免受过多细胞外基质积聚方面发挥着重要作用,如松弛素基因敲除小鼠随着年龄增长会出现广泛的纤维化就证明了这一点。松弛素通过抑制胶原蛋白生成同时促进其降解来减少因胶原蛋白过度沉积导致的瘢痕形成,并且在包括肝纤维化在内的几种细胞外基质相关疾病的动物模型中能减轻已形成的纤维化。用松弛素治疗可降低活化肝星状细胞的肌成纤维细胞表型,肝星状细胞是纤维化和肝硬化中主要的肝脏胶原蛋白产生细胞。松弛素还具有血流动力学效应,包括血管舒张,并且可以降低与肝硬化相关的门静脉高压。在本综述中,将简要概述肝纤维化和肝星状细胞的作用,随后介绍松弛素及其作用。还将讨论松弛素在治疗包括肝脏疾病在内的纤维化疾病临床前模型中的应用。最后,将描述松弛素在人类疾病中已完成、正在进行的临床试验,以及使用松弛素治疗疾病的局限性和未来方向。