Expression of expanded GGC repeats within NOTCH2NLC causes behavioral deficits and neurodegeneration in a mouse model of neuronal intranuclear inclusion disease

GGC repeat expansions within NOTCH2NLC have been identified as the genetic cause of neuronal intranuclear inclusion disease (NIID). To understand the molecular pathogenesis of NIID, here, we established both a transgenic mouse model and a human neural progenitor cells (hNPCs) model. Expression of th...

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Detalles Bibliográficos
Autores principales: Liu, Qiong, Zhang, Kailin, Kang, Yunhee, Li, Yangping, Deng, Penghui, Li, Yujing, Tian, Yun, Sun, Qiying, Tang, Yu, Xu, Keqin, Zhou, Yao, Wang, Jun-Ling, Guo, Jifeng, Li, Jia-Da, Xia, Kun, Meng, Qingtuan, Allen, Emily G., Wen, Zhexing, Li, Ziyi, Jiang, Hong, Shen, Lu, Duan, Ranhui, Yao, Bing, Tang, Beisha, Jin, Peng, Pan, Yongcheng
Formato: Online Artículo Texto
Lenguaje:English
Publicado: American Association for the Advancement of Science 2022
Materias:
Neuroscience
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9683706/
https://www.ncbi.nlm.nih.gov/pubmed/36417528
http://dx.doi.org/10.1126/sciadv.add6391

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9683706/
https://www.ncbi.nlm.nih.gov/pubmed/36417528
http://dx.doi.org/10.1126/sciadv.add6391

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