Mutant SOD1<ce:sup loc=post>G93A</ce:sup> triggers mitochondrial fragmentation in spinal cord motor neurons: Neuroprotection by SIRT3 and PGC-1α

@article{Song2013MutantSL,
  title={Mutant SOD1<ce:sup loc=post>G93A</ce:sup> triggers mitochondrial fragmentation in spinal cord motor neurons: Neuroprotection by SIRT3 and PGC-1α},
  author={Wenjun Song and Yuting Song and Brad Kincaid and Blaise Bossy and Ella Bossy-Wetzel},
  journal={Neurobiology of Disease},
  year={2013},
  volume={51},
  pages={72-81}
}
Mutations in the Cu/Zn Superoxide Dismutase (SOD1) gene cause an inherited form of ALS with upper and lower motor neuron loss. The mechanism underlying mutant SOD1-mediated motor neuron degeneration remains unclear. While defects in mitochondrial dynamics contribute to neurodegeneration, including ALS, previous reports remain conflicted. Here, we report an improved technique to isolate, transfect, and culture rat spinal cord motor neurons. Using this improved system, we demonstrate that mutant… CONTINUE READING

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