Electrophysiologic Biomarkers for Assessing Disease Progression and the Effect of Riluzole in SOD1 G93A ALS Mice

Electrophysiologic Biomarkers for Assessing Disease Progression and the Effect of Riluzole in SOD1 G93A ALS Mice
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DOI:
10.1371/journal.pone.0065976
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发表时间:
2013-06-06
期刊:
影响因子:
3.7
通讯作者:
Rutkove, Seward B.
Rutkove, Seward B.
中科院分区:
综合性期刊3区
文献类型:
--
作者:
Li, Jia;Sung, Minhee;Rutkove, Seward B.

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目的:比较电阻抗肌电图 (EIM) 50 kHz 相位与体重、运动评分、爪握耐力 (PGE)、CMAP 振幅和 MUNE,以识别 SOD1 G93A 小鼠的疾病进展和利鲁唑的作用。方法:23 只动物从 6 周龄开始在饮用水中接受 8 mg/kg/天的利鲁唑; 22只动物作为对照。每周进行体重、运动评分、PGE、CMAP、MUNE 和 EIM 以评估疾病进展。结果:治疗组和未治疗组之间没有发现临床疾病发作或生存率存在差异。此外,所有方法均未能确定利鲁唑的任何有益作用。因此,将所有动物的数据合并起来进行额外的分析。在 4 个参数中,EIM 阶段显示出相对于基线最早的变化以及整个测量期间最线性的下降。此外,EIM 相位与 PGE、CMAP 幅度和 MUNE 相关(Spearman r 分别 = 0.92、0.90 和 0.72,p
Objective: To compare electrical impedance myography (EIM) 50 kHz phase to weight, motor score, paw grip endurance (PGE), CMAP amplitude, and MUNE for the identification of disease progression and the effect of riluzole in the SOD1 G93A mouse.Methods: Twenty-three animals received 8 mg/kg/day riluzole in the drinking water starting at 6 weeks of age; 22 animals served as controls. Weight, motor score, PGE, CMAP, MUNE, and EIM were performed weekly to evaluate disease progression.Results: No difference in clinical disease onset or survival was found between treated and untreated groups. In addition, all methods failed to identify any beneficial effect of riluzole. Thus, data from all animals were combined for additional analyses. Of the 4 parameters, EIM phase showed the earliest change from baseline and the most linear decline throughout the entire measurement period. In addition, EIM phase correlated with PGE, CMAP amplitude, and MUNE (Spearman r = 0.92, 0.90, and 0.72, respectively, p