Mechanisms involved in spinal cord central synapse loss in a mouse model of spinal muscular atrophy.

@article{Tarabal2014MechanismsII,
  title={Mechanisms involved in spinal cord central synapse loss in a mouse model of spinal muscular atrophy.},
  author={Olga Tarabal and V{\'i}ctor Caraballo-Miralles and Andrea Cardona-Rossinyol and Francisco J. Correa and Gabriel Olmos and Jer{\`o}nia Llad{\'o} and Josep E. Esquerda and Jordi Calder{\'o}},
  journal={Journal of neuropathology and experimental neurology},
  year={2014},
  volume={73 6},
  pages={519-35}
}
Motoneuron (MN) cell death is the histopathologic hallmark of spinal muscular atrophy (SMA), although MN loss seems to be a late event. Conversely, disruption of afferent synapses on MNs has been shown to occur early in SMA. Using a mouse model of severe SMA (SMNΔ7), we examined the mechanisms involved in impairment of central synapses. We found that MNs underwent progressive degeneration in the course of SMA, with MN loss still occurring at late stages. Loss of afferent inputs to SMA MNs was… CONTINUE READING
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