亨廷顿病 (HD)是一种神经系统遗传病 ,疾病基因 -HD基因已被克隆 ,HD基因第一位外显子内含有CAG三核苷酸重复扩展序列 ,HD基因的 (CAG)n动态突变导致HD。但这种引起HD患者神经系统内特定区域内神经元核包涵体形成和变性脱失的发病机理仍不清楚 ,推测蛋白 -蛋白相互作用起着某种作用。近几年来开展了与HD基因产物huntingtin相互作用蛋白的研究 ,为阐明HD发病机理提供了新的概念 Huntington’s disease (HD) is a hereditary genetic disease of the nervous system. The disease gene-HD gene has been cloned. The first exon of the HD gene contains a CAG trinucleotide repeat extension sequence. The HD gene (CAG) n dynamic mutation Cause HD. However, the pathogenesis of CNS formation and degeneration of neurons in specific regions of the nervous system of HD patients remains unclear, suggesting that protein-protein interactions play a role. In recent years, research has been carried out on the protein interacting with huntingtin, an HD gene product, which provides a new concept for elucidating the pathogenesis of HD