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

bioRxiv (Cold Spring Harbor Laboratory) 2026 Preprint Open access

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Authors: Yalan Wan, Yilei Zheng, Chao Gao, Lu Yin, Fuze Zheng, Zhen Yu, Yi-Xiang Wang, Biyu Yang, Jie Zheng, Yun Yuan, Daojun Hong, Nicolas Charlet‐Berguerand
+3 more Jiaxi Yu, Zhaoxia Wang, Jianwen Deng
DOI: 10.64898/2026.02.10.705201
Cited by: 1
References: 30

Abstract

Summary 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.

Fields of Study & Subjects

Genetic Neurodegenerative Diseases Amyotrophic Lateral Sclerosis Research Parkinson's Disease Mechanisms and Treatments

Publication details

Journal / source
bioRxiv (Cold Spring Harbor Laboratory)
Publisher
openRxiv
Publication date
12 February 2026
Document type
Preprint