Ontology highlight
ABSTRACT:
SUBMITTER: Simon CM
PROVIDER: S-EPMC4972669 | biostudies-literature | 2016 Aug
REPOSITORIES: biostudies-literature
Simon Christian M CM Janas Anna M AM Lotti Francesco F Tapia Juan Carlos JC Pellizzoni Livio L Mentis George Z GZ
Cell reports 20160721 5
In spinal muscular atrophy, a neurodegenerative disease caused by ubiquitous deficiency in the survival motor neuron (SMN) protein, sensory-motor synaptic dysfunction and increased excitability precede motor neuron (MN) loss. Whether central synaptic dysfunction and MN hyperexcitability are cell-autonomous events or they contribute to MN death is unknown. We addressed these issues using a stem-cell-based model of the motor circuit consisting of MNs and both excitatory and inhibitory interneurons ...[more]