Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments.

@article{Swarup2011PathologicalHO,
  title={Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments.},
  author={Vivek Swarup and Daniel J. Phaneuf and Christine Bareil and Janice C. Robertson and Guy A Rouleau and Jasna Kriz and Jean-Pierre Julien},
  journal={Brain : a journal of neurology},
  year={2011},
  volume={134 Pt 9},
  pages={
          2610-26
        }
}
Transactive response DNA-binding protein 43 ubiquitinated inclusions are a hallmark of amyotrophic lateral sclerosis and of frontotemporal lobar degeneration with ubiquitin-positive inclusions. Yet, mutations in TARDBP, the gene encoding these inclusions are associated with only 3% of sporadic and familial amyotrophic lateral sclerosis. Recent transgenic mouse studies have revealed a high degree of toxicity due to transactive response DNA-binding protein 43 proteins when overexpressed under the… CONTINUE READING

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