Skeletal muscle-restricted expression of human SOD1 causes motor neuron degeneration in transgenic mice.

@article{Wong2010SkeletalME,
  title={Skeletal muscle-restricted expression of human SOD1 causes motor neuron degeneration in transgenic mice.},
  author={Margaret Fung-Yee Wong and Lee J. Martin},
  journal={Human molecular genetics},
  year={2010},
  volume={19 11},
  pages={2284-302}
}
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease of motor neurons (MNs) that causes skeletal muscle paralysis. Familial forms of ALS are linked to mutations in the superoxide dismutase-1 (SOD1) gene. The mechanisms of human SOD1 (hSOD1) toxicity to MNs are unknown. We hypothesized that skeletal muscle is a primary site of pathogenesis in ALS that triggers MN degeneration. We created transgenic (tg) mice expressing wild-type-, G37R- and G93A-hSOD1 gene variants only in… CONTINUE READING

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