uN2CpolyG-mediated p65 nuclear sequestration suppresses the NF-κB-NLRP3 pathway in neuronal intranuclear inclusion disease

uN2CpolyG-mediated p65 nuclear sequestration suppresses the NF-κB-NLRP3 pathway in neuronal intranuclear inclusion disease

Abstract Background Neuronal intranuclear inclusion disease (NIID) is genetically linked to CGG repeat expansion in the 5’-untranslated region of the NOTCH2NLC gene, with nascent polyglycine-containing protein (uN2CpolyG) identified as a primary pathogenic factor. Emerging clinical evidence suggests...

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Bibliographic Details
Main Authors: Yu Shen, Kaiyan Jiang, Dandan Tan, Min Zhu, Yusen Qiu, Pencheng Huang, Wenquan Zou, Jianwen Deng, Zhaoxia Wang, Ying Xiong, Daojun Hong
Format: Article
Language:English
Published: BMC 2025-02-01
Series:Cell Communication and Signaling
Subjects:
Neuronal intranuclear inclusion disease
CGG repeat expansion
NF-κB pathway
NLRP3 inflammasome
Autophagy
Single-cell sequencing
Online Access:https://doi.org/10.1186/s12964-025-02079-1
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