This strain is an hHTT gene knock-in mouse model generated by gene editing technology, in which a mutated human HTT gene sequence carrying over 100 CAG repeats is inserted into the mouse genome. Mice carrying these mutations have been reported to exhibit pathological characteristics and functional impairment phenotypes of Huntington’s disease and can be used for the development and screening of therapeutic drugs for Huntington's disease as well as for safety evaluation. The detection data shows that this model can successfully express the mutated human HTT gene and protein.