Article
Survival motor neuron protein in motor neurons determines synaptic integrity in spinal muscular atrophy.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 20 Jun 2012
Martinez Tara L, Kong Lingling, Wang Xueyong, Osborne Melissa A, Crowder Melissa E, Van Meerbeke James P, Xu Xixi, Davis Crystal, Wooley Joe, Goldhamer David J, Lutz Cathleen M, Rich Mark M, Sumner Charlotte J
Abstract excerpt
The inherited motor neuron disease spinal muscular atrophy (SMA) is caused by deficient expression of survival motor neuron (SMN) protein and results in severe muscle weakness. In SMA mice, synaptic dysfunction of both neuromuscular junctions (NMJs) and central sensorimotor synapses precedes motor neuron cell death. To address whether this synaptic dysfunction is due to SMN deficiency in motor neurons, muscle, or...
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