Impaired Wheel Running Exercise in CLC-1 Chloride Channel-Deficient Myotonic Mice.
Impaired Wheel Running Exercise in CLC-1 Chloride Channel-Deficient Myotonic Mice.
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DOI：
10.3389/fphys.2011.00047
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发表时间：
2011
影响因子：
4
通讯作者：
Pollarine J
中科院分区：
文献类型：
--
作者：
van Lunteren E;Moyer M;Cooperrider J;Pollarine J
背景：肌肉ClC-1氯通道的遗传缺陷导致肌强直，最突出的表现是肌肉松弛减慢。人类在开始收缩时会经历肌肉僵硬，尽管这可以通过反复努力来克服(“热身”现象)。ClC-1缺乏在多大程度上损害运动活动是有争议的。我们假设骨骼肌ClC-1氯通道缺陷会导致自发运动的严重减少。方法/主要发现：为了定量检测这一点，给强直性ClC-1缺陷小鼠提供了使用跑轮的通道，随后对它们的自发跑步活动进行了量化。强直性肌强直小鼠与正常小鼠之间的跑步差异不是在安装跑轮后即刻出现的，而是在第2周完全建立起来的。在第8周，强直性肌强直小鼠的跑步次数显著少于正常小鼠(322 ± 177vs5058 ± 1253 m/d，P = 0.025)。此外，与正常动物相比，强直性肌强直动物的连续跑步时间(18.8 ± 1.5vs59.0 ± 3.7 min，P <； 0.001)和每次连续跑步的距离(58 ± 38vs601 ± 174 m，P = 0.048)显著减少。结论/意义：这些结果表明ClC-1缺乏性肌强直小鼠的自发运动活动明显减弱，总距离和连续跑次数均减少。
Background: Genetic deficiency of the muscle CLC-1 chloride channel leads to myotonia, which is manifested most prominently by slowing of muscle relaxation. Humans experience this as muscle stiffness upon initiation of contraction, although this can be overcome with repeated efforts (the “warm-up” phenomenon). The extent to which CLC-1 deficiency impairs exercise activity is controversial. We hypothesized that skeletal muscle CLC-1 chloride channel deficiency leads to severe reductions in spontaneous exercise. Methodology/Principal Findings: To examine this quantitatively, myotonic CLC-1 deficient mice were provided access to running wheels, and their spontaneous running activity was quantified subsequently. Differences between myotonic and normal mice in running were not present soon after introduction to the running wheels, but were fully established during week 2. During the eighth week, myotonic mice were running significantly less than normal mice (322 ± 177 vs 5058 ± 1253 m/day, P = 0.025). Furthermore, there were considerable reductions in consecutive running times (18.8 ± 1.5 vs 59.0 ± 3.7 min, P < 0.001) and in the distance per consecutive running period (58 ± 38 vs 601 ± 174 m, P = 0.048) in myotonic compared with normal animals. Conclusion/Significance: These findings indicate that CLC-1 chloride deficient myotonia in mice markedly impairs spontaneous exercise activity, with reductions in both total distance and consecutive running times.
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DOI：
10.1073/pnas.85.11.3880
发表时间：
1988-06-01
影响因子：
11.1
作者：
FUCHTBAUER, EM;REININGHAUS, J;JOCKUSCH, H
通讯作者：
JOCKUSCH, H
影响因子：
3.4
作者：
Hara, H;Nolan, PM;Fischbeck, KH
通讯作者：
Fischbeck, KH
影响因子：
3.4
作者：
MEHRKE, G;BRINKMEIER, H;JOCKUSCH, H
通讯作者：
JOCKUSCH, H
影响因子：
3.5
作者：
Agbulut, O;Noirez, P;Jockusch, H
通讯作者：
Jockusch, H
影响因子：
3.9
作者：
Pusch, M
通讯作者：
Pusch, M