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bioRxiv · 10.64898/2026.02.10.705201

A novel uN2CpolyG Transgenic Mouse Model Recapitulates Multisystemic polyG Proteinopathy Pathology of Neuronal Intranuclear Inclusion Disease

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.
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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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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.. 2026-02-12. A novel uN2CpolyG Transgenic Mouse Model Recapitulates Multisystemic polyG Proteinopathy Pathology of Neuronal Intranuclear Inclusion Disease. https://doi.org/10.64898/2026.02.10.705201

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