Method for constructing mouse model of neuronal intranuclear inclusion disease and application thereof

CN118633572BActive Publication Date: 2026-07-21BEIJING GENECRADLE PHARM CO LTD
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Patent Information

Authority / Receiving Office
CN · China
Patent Type
Patents(China)
Current Assignee / Owner
BEIJING GENECRADLE PHARM CO LTD
Filing Date
2023-03-06
Publication Date
2026-07-21

AI Technical Summary

Technical Problem

The lack of stable and efficient mouse models in the current technology for studying GGC duplication impairment related to the NOTCH2NLC gene, especially intranuclear inclusion body disease (NIID), has affected the research on the pathogenesis of this disease and the development of treatment methods.

Method used

AAV vectors were constructed by inserting a polynucleotide sequence encoding 50–200 glycine residues upstream of exon 1 of the NOTCH2NLC transcript 2 into a mouse model, and mice were infected by intracerebral administration to establish a mouse model of NIID disease.

Benefits of technology

A stable mouse model capable of forming a large number of p62-positive inclusion bodies in the neuronal cell nucleus was obtained, providing a tool for studying the pathogenesis of NIID and developing therapeutic drugs.

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Abstract

The present application provides a method for constructing a mouse model stably expressing neuronal nuclear inclusion disease (NIID) and its use. Specifically, the present application relates to constructing a mouse model expressing NIID disease by means of an AAV packaging system stably expressing mutant N2C-iso2 protein (PolyG_N2C-iso2) in the brain. The present application also relates to the use of the mouse model in studying the pathogenesis of NIID and developing drugs for treating NIID disease.
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