This strain is a mouse Fus gene humanized model and can be used for research on ALS. The homozygous B6-hFUS mice are viable and fertile. In addition, based on the independently developed TurboKnockout fusion BAC recombination technology, Cyagen can also generate hot mutation models based on this strain (e.g. FUS (p.R521C)) and provide customized services for specific mutations to meet the experimental needs in pharmacology and other fields related to ALS.