The GPT hSOD1 G93A mouse model can recapitulate progressive ALS-like phenotypes

Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disease that lacks effective therapeutic strategies to date. GemPharmatech (GPT) generated the GPT hSOD1 G93A transgenic mouse strain to recapitulate ALS-like phenotypes in an animal model, in support of medical research and drug development. The hSOD1 G93A mouse model carries the G93A mutation in human SOD1 gene, which is widely expressed in mice. We observed an apparent body weight decrease in 25-week-old mice. We further performed a range of behavioral tests to identify the disease onset timing in GPT hSOD1 G93A mouse model, revealing that male mice experienced onset at 25 weeks while female mice experienced onset about 2 weeks later. Pathological findings demonstrate gastrocnemius muscle degeneration and detectable immune cell infiltration following disease onset. We detected transcriptome alterations in the spinal cord tissue of GPT hSOD1 G93A mice and found that immune responses were activated after disease onset. Finally, the mice can survive for up to 8 months. Our data suggest that the GPT hSOD1 G93A transgenic mouse model can serve as a crucial tool for neurodegenerative disease research, providing valuable insights into disease mechanisms and supporting the development of disease-modifying therapies for ALS.