The authors show that in a mouse model of spinal muscular atrophy (SMA), there is a reduction in sensory synaptic drive that leads to motor neuron dysfunction and motor behavior impairments. SMA motor neurons showed a lower surface expression of Kv2.1 potassium channels and reduced spiking ability. Increasing neuronal activity pharmacologically led to the normalization of Kv2.1 surface expression and an improvement in motor function.
- Emily V Fletcher
- Christian M Simon
- George Z Mentis
