The wobbler mouse: a neurodegeneration jigsaw puzzle.

  title={The wobbler mouse: a neurodegeneration jigsaw puzzle.},
  author={S{\'e}verine Boill{\'e}e and Marc Peschanski and Marie-Pierre Junier},
  journal={Molecular neurobiology},
  volume={28 1},
Various mutations in humans and animals lead to the selective and progressive degeneration of motoneurons, resulting in muscular weakness, subsequent paralysis, and death (1-3). Amyotrophic lateral sclerosis (ALS) is the most common adult human motoneuron disease, but the vast majority of sporadic and familial cases of ALS are still of unknown origin (4). Murine models of motoneuron diseases, derived from spontaneous mutations in the colonies, have been known for half a century. Prior to the… CONTINUE READING
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