bioRxivAnimal Behavior & CognitionPreprint
A novel uN2CpolyG Transgenic Mouse Model Recapitulates Multisystemic polyG Proteinopathy Pathology of Neuronal Intranuclear Inclusion Disease
Yalan Wan, Yilei ZhengiD, Chao Gao, Yuxuan Lu, Fuze ZhengiD, Zhen Yu, Jiaxin WangiD, Biyu Yang, Jie Zheng, Yun YuaniD, Daojun HongiD, Nicolas Charlet‐BerguerandiD, Jiaxi YuiD, Zhaoxia WangiD, Jianwen DengiD
Abstract
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.
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