Exact(3)
Supplementary Figure 7 Integrate-and-fire motor neuron model.
"... To explore the relationship between conductances, and in particular how they influence the activity of motor neurons in the well characterized leech heartbeat system, we developed a new multi-compartmental Hodgkin-Huxley style leech heart motor neuron model.
Interestingly, in the motor neuron model, maturation is accompanied with an increase of XIAP and a decrease in its inhibitor XIAP-associated factor (XAF) levels.
Similar(57)
Looking at our motor neuron models, we observed that GE reduced the paralysis and neuronal degeneration phenotypes caused by the expression of mutant mTDP-43 or mFUS and reduced the fraction of insoluble mutant proteins (Fig. 1D I), suggesting that GE may reduce the cellular load of toxic misfolded proteins.
It was observed in a study using motor neuron models, in cultures containing mutant SOD1G93A, through administration of adenovirus vector, that VEGF increased neurons survival [ 215].
Motor neuron population model.
These data represent an unprecedented therapeutic success in motor neuron disease models compared with other pharmacological/molecular treatments.
This conclusion is supported by the strategy taken by Gross and colleagues (2007) who used lentiviral vectors to reduce RHOA expression and promote regeneration of motor neuron in models of spinal cord ischaemia.
We used Drosophila, which encodes a single SMN ortholog, survival motor neuron (Smn), to model SMA, since reduction of Smn function leads to defects that mimic the SMA pathology in humans.
Movement speed may be a particularly sensitive index of early motor deterioration in ALS as the disease appears to selectively affect fast-twitch muscles, as noted in early phases of the motor neuron disease mouse model when the animal is presymptomatic (49, 50).
Moreover, several studies have demonstrated that astrocyte-specific overexpression of Nrf2, an antioxidant transcription factor, reduces chemical-mediated neurotoxicity modeling PD and Huntington's disease, as well as genetically induced motor neuron degeneration in models of amyotrophic lateral sclerosis (ALS).
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