The Alteration of Intrinsic Excitability and Synaptic Transmission in Lumbar Spinal Motor Neurons and Interneurons of Severe Spinal Muscular Atrophy Mice
Abstract
Spinal muscular atrophy (SMA) is the leading genetic cause of death in infants. Studies with mouse models have demonstrated increased excitability and loss of afferent proprioceptive synapses on motor neurons (MNs). To further understand functional changes in the motor neural network occurring in SMA, we studied the intrinsic excitability and synaptic transmission of both MNs and interneurons (INs) from ventral horn in the lumbar spinal cord in the survival motor neuron (SMN)Δ7 mouse model. We found significant differences in the membrane properties of MNs in SMA mice compared to littermate controls, including hyperpolarized resting membrane potential, increased input resistance and decreased membrane capacitance. Action potential (AP) properties in MNs from SMA mice were also different from controls, including decreased rheobase current, increased amplitude and an increased afterdepolarization (ADP) potential. The relationship between AP firing frequency and injected current was reduced in MNs, as was the threshold current, while the percentage of MNs showing long-lasting potentiation (LLP) in the intrinsic excitability was higher in SMA mice. INs showed a high rate of spontaneous firing, and those from SMA mice fired at highe...Continue Reading
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