基于中医“虚、痰瘀、毒”理论探讨铜稳态失调诱导调节性细胞死亡在肝豆状核变性中的作用

Exploration on the Role of Regulatory Cell Death Induced by Dysregulation ofCopper Homeostasis in Wilson's Disease Based on “Deficiency, Phlegmand Blood Stasis, and Toxin” of Traditional Chinese Medicine

肝豆状核变性(WD)是一种铜代谢障碍的染色体隐性遗传代谢性疾病,其中医核心病机为“虚、痰瘀、毒”。铜稳态失调诱导调节性细胞死亡包括多种生物学途径,在WD的发生、发展中起到了核心作用,也是WD“虚、痰瘀、毒”中医病机的微观体现。本课题组在前期研究基础上,以“补脾益肾治其本,化痰祛瘀,解毒通络治其标”为治则,从铜稳态失调诱导调节性细胞死亡机制出发,进一步探究中医药治疗WD的机制,为中西医结合治疗WD提供新的思路。

Wilson's disease(WD)is an autosomal recessive genetic disease with copper metabolism disorder.The core pathogenesis of WD in traditional Chinese medicine(TCM)is“deficiency,phlegm and blood stasis,and toxin”.Regulatory cell death induced by dysregulation of copper homeostasis is through multiple biological pathways,which plays a central role in the occurrence and development of WD,and is also a microcosmic manifestation of the TCM pathogenesis of“deficiency,phlegm and blood stasis,and toxin”of the disease.On the basis of previous research,taking“tonifying spleen and replenishing kidney should be performed in order to treat the root,resolving phlegm and dispelling stasis,and detoxication and unblocking collaterals should be performed to treat the tip”as treatment principle,and starting from the mechanism of copper homeostasis imbalance inducing regulatory cell death,this research group further explored the mechanism of traditional Chinese medicine in treating WD,providding new ideas for the combination of traditional Chinese and Western medicine in the treatment of the disease.