Motor dysfunction and neurodegeneration in a C9orf72 mouse line expressing poly-PR
oleh: Zongbing Hao, Liu Liu, Zhouteng Tao, Rui Wang, Haigang Ren, Hongyang Sun, Zixuan Lin, Zhixiong Zhang, Chenchen Mu, Jiawei Zhou, Guanghui Wang
| Format: | Article |
|---|---|
| Diterbitkan: | Nature Portfolio 2019-07-01 |
Deskripsi
Repeat-associated non-ATG translation of dipeptide repeat proteins (DPRs) contributes to disease manifestation in FTD/ALS associated with the hexanucleotide repeat expansion of the C9ORF72 gene. Here the authors show that a transgenic mouse line expressing poly-PR28 in neurons displays some signs of neuronal loss that mirrors that seen in the disease.