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Of these, only 2 were statistically up-regulated in SMAr motor neuron compared to WTr (Fig. 9a).
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14 Markedly elevated CSF NfL levels in ALS may be explained by the higher content of axonal proteins in motor neurons compared to other neuronal populations.
For example, there appear to be less defined D169G puncta in primary motor neurons compared with other variants, suggesting that the association with neuronal RNA granules is dependent on a fully functional RRM1 domain.
535 downregulated genes (green) and 364 upregulated genes (red) were found in SOD1 +/A272C motor neurons compared with its isogenic control motor neurons.
Patient stem cell clones produced a similar number of motor neurons compared to controls, with or without androgen treatment.
Treatment with SUN N8075 (30 mg/kg, s.c./day) in the G93A mice significantly increased the number of surviving motor neurons compared with that in the vehicle-treated G93A mice (Fig. 5A).
Furthermore, they appear much more pronounced in motor neurons compared with sensory neurons.
Interestingly, there remained a trend for increased DNA repair in SMAr motor neurons compared to SMAv.
Comparative analysis in ArrayAssist® identified 1170 transcripts differentially expressed in SOD1-related motor neurons compared with control motor neurons, of which 524 were increased and 646 were decreased in mt SOD1 motor neurons compared with controls.
We therefore looked for transcriptional changes that occurred in SMAv motor neurons compared to SMAr and compared to WTv.
Of particular note, 5 of these transcripts were up-regulated in SMAv motor neurons compared to SMAr (Fig. 9a).
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