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Thus, there is gradual return of normal motor unit output with time after SMN restoration.
This suggests that in early clinical trials the disease progression (i.e., motor unit loss) may be halted with SMN therapies but development of improved motor unit output (and thus improvement of functional outcomes) may be more gradual or delayed.
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Thus, both the recruitment and the activity pattern of each motor unit vary with the force output.
40, 41 Importantly, in the mouse this exuberant innervation is functional and increases synaptic output of each motor unit 10-fold at birth.
The potential of these measures includes the ability to assess the output of the motor unit in vivo and the ability to provide parallel measures in mouse and human and thus predict outcomes in early clinical trials.
We speculate that G/A subjects show greater motor unit efficiency with increasing levels of force output than G/G individuals.
Furthermore, our MUNE results demonstrate an expansion of individual motor unit territory in the SMNΔ7 mice that leads to each motor unit representing a larger percentage of the total output.
Rate coding governs motor unit firing.
The functional unit of muscle contraction is the motor unit.
Figure 1: Single motor unit action potential (MUAP).
CT assessment of motor unit action potentials.
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