Nature Communications (Jul 2019)

Motor dysfunction and neurodegeneration in a C9orf72 mouse line expressing poly-PR

  • Zongbing Hao,
  • Liu Liu,
  • Zhouteng Tao,
  • Rui Wang,
  • Haigang Ren,
  • Hongyang Sun,
  • Zixuan Lin,
  • Zhixiong Zhang,
  • Chenchen Mu,
  • Jiawei Zhou,
  • Guanghui Wang

DOI
https://doi.org/10.1038/s41467-019-10956-w
Journal volume & issue
Vol. 10, no. 1
pp. 1 – 11

Abstract

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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.