Transcriptional profile of primary astrocytes expressing ALS-linked mutant SOD1.

  title={Transcriptional profile of primary astrocytes expressing ALS-linked mutant SOD1.},
  author={Marcelo R. Vargas and Mariana Pehar and Pablo J D{\'i}az-Amarilla and Joseph S. Beckman and Luis H. Barbeito},
  journal={Journal of neuroscience research},
  volume={86 16},
Amyotrophic lateral sclerosis (ALS) is caused by the progressive degeneration of motor neurons. Mutations in the Cu/Zn superoxide dismutase (SOD1) are found in approximately 20% of patients with familial ALS. Mutant SOD1 causes motor neuron death through an acquired toxic property. Although the molecular mechanism underlying this toxic gain-of-function remains unknown, evidence support the role of mutant SOD1 expression in nonneuronal cells in shaping motor neuron degeneration. We have… CONTINUE READING


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