SOD1 G93A 转基因小鼠的神经肌肉传递缺陷在人脐带血细胞给药后得到改善。
Defective neuromuscular transmission in the SOD1 G93A transgenic mouse improves after administration of human umbilical cord blood cells.
机构信息
Department of Neurology and Neurosciences, New Jersey Medical School-UMDNJ, 185 South Orange Avenue, Newark, NJ 07103-2714, USA.
出版信息
Stem Cell Rev Rep. 2012 Mar;8(1):224-8. doi: 10.1007/s12015-011-9281-3.
To assess the effect of human umbilical cord blood (hUCB) transplantation on neuromuscular transmission in SOD1(G93A) transgenic mice, we studied the probability of neuromuscular transmission (PNMT), a relevant physiological indicator of motor nerve function, in 3 SOD1(G93A) mice transplanted with hUCB and compared to PNMT in 4 SOD1(G93A) 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 SOD1(G93A) 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 SOD1(G93A) 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 SOD1(G93A) mice. Testing PNMT in the SOD1(G93A) mouse model could be used as a simple in vitro protocol to detect a positive cellular response to therapeutic interventions in ALS.
为了评估人脐血（hUCB）移植对 SOD1（G93A）转基因小鼠神经肌肉传递的影响，我们研究了神经肌肉传递的概率（PNMT），这是运动神经功能的一个相关生理指标，在 3 只接受 hUCB 移植的 SOD1（G93A）小鼠中进行了研究，并与 4 只未进行细胞移植的 SOD1（G93A）小鼠和 3 只非突变 SOD1 转基因小鼠的 PNMT 进行了比较。对于从非突变 SOD1 转基因小鼠分离的制剂，PNMT 在 70 和 90 Hz 刺激的前 5 秒分别为 0.93 和 0.84。PNMT 在刺激结束时逐渐下降至 0.77 和 0.42。相比之下，未经处理的突变 SOD1（G93A）小鼠的制剂在 70 和 90 Hz 刺激的前 5 秒的 PNMT 分别为 0.52 和 0.36（p<0.05）。hUCB 的治疗显著（p<0.05）改善了 SOD1（G93A）制剂的 PNMT。也就是说，初始 5 秒的 PNMT 分别为 70 和 90 Hz 刺激的 0.88 和 0.68。我们得出结论，hUCB 移植显著改善了 SOD1（G93A）小鼠肌肉的 PNMT。在 SOD1（G93A）小鼠模型中测试 PNMT 可以作为一种简单的体外方案，用于检测对 ALS 治疗干预的阳性细胞反应。
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