Defective Neuromuscular Transmission in the SOD1G93A Transgenic Mouse Improves After Administration of Human Umbilical Cord Blood Cells
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- Souayah, N., Coakley, K.M., Chen, R. et al. Stem Cell Rev and Rep (2012) 8: 224. doi:10.1007/s12015-011-9281-3
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To assess the effect of human umbilical cord blood (hUCB) transplantation on neuromuscular transmission in SOD1G93A transgenic mice, we studied the probability of neuromuscular transmission (PNMT), a relevant physiological indicator of motor nerve function, in 3 SOD1G93A mice transplanted with hUCB and compared to PNMT in 4 SOD1G93A mice without cell transplantation and 3 non-mutant SOD1 transgenic mice. For preparations isolated from non-mutant SOD1 transgenic mice, PNMT was 0.93 and 0.84 during the first 5 s of 70 and 90 Hz trains, respectively. PNMT gradually declined to 0.77 and 0.42 at the end of the trains. In striking contrast, PNMT for preparations from non-treated mutant SOD1G93A mice was 0.52 and 0.36 in the first 5 s of 70 and 90 Hz trains, respectively (p < 0.05). Treatment with hUCB significantly (p < 0.05) improved PNMT in SOD1G93A preparations. That is, the initial 5 s PNMT was 0.88 and 0.68 for the 70 and 90 Hz stimuli, respectively. We concluded that hUCB transplantation significantly improved PNMT for muscles removed from SOD1G93A mice. Testing PNMT in the SOD1G93A mouse model could be used as a simple in vitro protocol to detect a positive cellular response to therapeutic interventions in ALS.