Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats.

  title={Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats.},
  author={Cao Huang and Jianbin Tong and Fangfang Bi and Hongxia Zhou and Xu-Gang Xia},
  journal={The Journal of clinical investigation},
  volume={122 1},
Amyotrophic lateral sclerosis (ALS) is characterized by progressive motor neuron degeneration, which ultimately leads to paralysis and death. Mutation of TAR DNA binding protein 43 (TDP-43) has been linked to the development of an inherited form of ALS. Existing TDP-43 transgenic animals develop a limited loss of motor neurons and therefore do not faithfully reproduce the core phenotype of ALS. Here, we report the creation of multiple lines of transgenic rats in which expression of ALS… CONTINUE READING
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