Objective       To establish a transgenic mice expressing a new human mutant SOD1 gene (mSOD1

GenBank ID EF143990)

observe the clinical phenotype of this mice model

and determine the relationship of the gene with amyotrophic lateral sclerosis (ALS).       Methods        A PCI plasmid carrying mSOD1 gene was constructed

and was microinjected into the pronuclei of fertilized mouse egg. Born offspring of these mice were identified by PCR. The clinical phenotypes of the mice were experimented by rota-rod test and footprint analysis.       Results       PCR results confirmed that the transgenic mice expressing mSOD1 gene were established successfully. Behavioral testing showed that the lasting time staying on the wheel was significantly shorter in the transgenic mice than the wild-type ones (59.17±26.27 vs 171.83±20.98 s

P0.01). The transgenic mice showed double-leg lameness and drag in 8 months after born and impaired walking patterns in footprint. Hind limb extension was significantly shorter in the transgenic mice than the wild-type ones (34.83±9.72 vs 52.78±7.22 mm

P0.01).       Conclusion       An mSOD1 transgenic mice model is successfully established

and presents similar clinical phenotype of ALS.