Wilson病(Wilson disease,WD)肝纤维化引起的肝功能衰竭是WD患者死亡主要原因，是脑病与肝病学科交叉研究的难点。课题组前期对WD肝纤维化进行相关研究，发现肝豆灵有干预p38MAPK信号通路诱导肝星状细胞凋亡的作用，对WD代谢产物有调节效应。骨髓间充质干细胞(BMSCs)是肝纤维化研究的新手段，肝再生是肝纤维化防治的新靶点，但肝豆灵促进BMSCs干预WD肝纤维化、调控肝再生机制尚不明确。根据WD肝纤维化“肾精亏虚、痰瘀阻络”病机及BMSCs中医“肾精”属性，课题组假设：补肾活血化浊法既符合WD肝纤维化中医病机，同时与BMSCs中医内涵相切合，肝豆灵能够促进BMSCs归巢，调控肝再生，治疗肝纤维化。本课题继续以p38MAPK信号通路为切入点，代谢组学为研究工具，将中医药基础研究与干细胞技术有机结合，寻找中药复方与干细胞结合的靶点，为疑难疾病WD肝纤维化寻求新的治疗方法。

Liver failure induced by hepatic fibrosis is the main cause of death in patients with Wilson disease,it is the difficulty point of encephalopathy and liver disease.The study group carried on the related research in early stage to WD hepatic fibrosis,found that Gandouling can interfere with the expression of p38MAPK signaling pathway and induced the apoptosis of hepatic stellate cells ,and it has obvious regulation effect on WD marker metabolites.BMSCs is a new methods in the study of hepatic fibrosis,liver regeneration is a new target for prevention and treatment of hepatic fibrosis.However, it is not clear that the mechanism of Gandouling promoting the BMSCs to intervence liver regeneration .According to the pathogenesis of kidney essence deficiency, phlegm and blood stasis blocking collaterals of WD hepatic fibrosis , and the TCM attribute of kidney essence of BMSCs, so we think,tonifying kidney and promoting blood circulation and removing turbidity, which is not only consistent with the TCM pathogenesis of WD hepatic fibrosis,but also agree with the TCM connotation of BMSCs,Gandouling can promote the homing of BMSCs, regulate the liver regeneration, treatment the hepatic fibrosis. This subject continue to take the p38MAPK signaling pathway as the breakthrough point, metabonomics as the research tool,combining traditional Chinese medicine research with stem cell technology,seeking for the target of combining traditional Chinese medicine compound with stem cells,to find a new therapeutic method for the difficult disease of hepatic fibrosis in WD.