Stem Cell Reviews and Reports

, Volume 8, Issue 1, pp 224–228 | Cite as

Defective Neuromuscular Transmission in the SOD1G93A Transgenic Mouse Improves After Administration of Human Umbilical Cord Blood Cells

  • Nizar Souayah
  • K. M. Coakley
  • R. Chen
  • Norman Ende
  • Joseph J. McArdle


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.


Human umbilical cord blood Amyotrophic lateral sclerosis SOD1 G93A Transplantation Neuromuscular transmission 



Supported by the Abraham S. Ende Research Foundation, The Kirby Foundation, The Toohey Neuroscience Fund at New Jersey Medical School and NINDS NS 045979.


The authors indicate no potential conflicts of interest.


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Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Nizar Souayah
    • 1
    • 4
  • K. M. Coakley
    • 2
  • R. Chen
    • 3
  • Norman Ende
    • 3
  • Joseph J. McArdle
    • 2
  1. 1.Department of Neurology and NeurosciencesNew Jersey Medical School-UMDNJNewarkUSA
  2. 2.Department of Pharmacology & PhysiologyNew Jersey Medical School-UMDNJNewarkUSA
  3. 3.Department of Pathology & Laboratory MedicineNew Jersey Medical School-UMDNJNewarkUSA
  4. 4.NewarkUSA

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