肝豆状核变性，又名Wilson病（WD），是一种常染色体隐性遗传的慢性进行性铜代谢障碍性疾病。因个体的差异, 出现复杂多样的临床表现,但无论是脑型、肝型还是其他类型,所有患者均表现有不同程度的肝脏慢性损害。肝纤维化几乎是每一个 WD患者肝脏的主要病理改变，也是导致WD死亡的一个重要原因。因此重视对早期肝硬化特别是其前期病理阶段肝纤维化的早期发现及干预治疗研究具有十分重要的临床意义。本课题在前期研究的基础上，以理想的WD模型 TX小鼠作为研究对象，在肝豆扶木汤不同浓度干预下，分别从血清学、病理形态学、分子生物学以及肝星状细胞增殖、凋亡等进行动态观察，通过TGF-β1/Smad信号通路探讨肝豆扶木汤逆转肝纤维化的作用及机制。

Hepatolenticular degeneration，also called Wilson disease，is an autosomal recessive genetic、chronic and progressive disorder in which is caused by copper metabolism.Because of individual difference， patients have complicated and diverse clinical manifestations。However,all the patients perform varying degrees of chronic liver damage ，in spite of who are cerebral type、hepatic type or other types。Hepatic fibrosis is the main pathological changes in the liver of almost every WD patient and also the main cause of death。Therefore,it is very meaningful and important to detect early and interfere with early cirrhosis of the liver，especially the prior pathological stage -liver fibrosis。Based on previous studies，We will observe serological index 、morphology index 、molecular biology index and proliferation 、apoptosis of Hepatic stellate cells dynamically towards the ideal model TX mice by different concentrations of Goufumutang（GDFMT） in order to explore GDFMT reverses mechanism of hepatic cirrhosis on WD by TGF-β1/Smad signaling pathway in this study。