Wan, Y.; Zheng, Y.; Gao, C.; Lu, Y.; Zheng, F.; Yu, Z.; Wang, J.; Yang, B.; Zheng, J.; Yuan, Y.; Hong, D.; Charlet-Berguerand, N.; Yu, J.; Wang, Z.; Deng, J.

Neuronal intranuclear inclusion disease (NIID) is a polyglycine disease that primarily affects the neuronal and neuromuscular systems. Here, we developed a novel transgenic mouse model that faithfully recapitulates the multisystemic impairments associated with polyG intranuclear inclusions. Our findings demonstrate that polyG expression induces neurodegeneration, behavioral deficits, and age-dependent accumulation of uN2CpolyG aggregates across multiple tissues.