Necroptosis Drives Motor Neuron Death in Models of Both Sporadic and Familial ALS

  title={Necroptosis Drives Motor Neuron Death in Models of Both Sporadic and Familial ALS},
  author={Diane B Re and Virginia Le Verche and Changhao Yu and Mackenzie Weygandt Mathis and Kristin A. Politi and Sudarshan Phani and Burcin Ikiz and Lucas Barbosa Hoffmann and Martijn Koolen and Tetsuya Nagata and Dimitra Papadimitriou and P{\'e}ter Nagy and Hiroshi Mitsumoto and Shingo Kariya and Hynek Wichterle and C. E. Henderson and Serge E. Przedborski},
Most cases of neurodegenerative diseases are sporadic, hindering the use of genetic mouse models to analyze disease mechanisms. Focusing on the motor neuron (MN) disease amyotrophic lateral sclerosis (ALS), we therefore devised a fully humanized coculture model composed of human adult primary sporadic ALS (sALS) astrocytes and human embryonic stem-cell-derived MNs. The model reproduces the cardinal features of human ALS: sALS astrocytes, but not those from control patients, trigger selective… CONTINUE READING
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