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Myotonia. An evaluation of the chloride hypothesis.肌强直。氯化物假说的评估。
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BK channels promote action potential repolarization in skeletal muscle but contribute little to myotonia.BK 通道促进骨骼肌动作电位复极化,但对肌强直的贡献很小。
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本文引用的文献

1
A quantitative description of membrane current and its application to conduction and excitation in nerve.膜电流的定量描述及其在神经传导和兴奋中的应用。
J Physiol. 1952 Aug;117(4):500-44. doi: 10.1113/jphysiol.1952.sp004764.
2
Sodium, potassium, and chloride fluxes in intercostal muscle from normal goats and goats with hereditary myotonia.正常山羊和患有遗传性肌强直的山羊肋间肌中的钠、钾和氯通量。
J Gen Physiol. 1966 Sep;50(1):89-111. doi: 10.1085/jgp.50.1.89.
3
Cable properties of external intercostal muscle fibres from myotonic and nonmyotonic goats.强直性山羊和非强直性山羊肋间外肌纤维的电缆特性
J Physiol. 1969 Oct;204(3):539-50. doi: 10.1113/jphysiol.1969.sp008930.
4
Voltage clamp experiments in striated muscle fibres.横纹肌纤维的电压钳实验。
J Physiol. 1970 Jul;208(3):607-44. doi: 10.1113/jphysiol.1970.sp009139.
5
The kinetics of mechanical activation in frog muscle.青蛙肌肉中机械激活的动力学。
J Physiol. 1969 Sep;204(1):207-30. doi: 10.1113/jphysiol.1969.sp008909.
6
Cable parameters, sodium, potassium, chloride, and water content, and potassium efflux in isolated external intercostal muscle of normal volunteers and patients with myotonia congenita.正常志愿者和先天性肌强直患者离体肋间外肌的电缆参数、钠、钾、氯和水含量以及钾外流情况。
J Clin Invest. 1971 Oct;50(10):2091-103. doi: 10.1172/JCI106703.
7
Reconstruction of the action potential of frog sartorius muscle.青蛙缝匠肌动作电位的重建。
J Physiol. 1973 Nov;235(1):103-31. doi: 10.1113/jphysiol.1973.sp010380.
8
On the repetitive discharge in myotonic muscle fibres.关于强直性肌纤维的重复放电。
J Physiol. 1974 Jul;240(2):505-15. doi: 10.1113/jphysiol.1974.sp010620.
9
Changes of action potential shape and velocity for changing core conductor geometry.改变核心导体几何形状时动作电位形状和速度的变化。
Biophys J. 1974 Oct;14(10):731-57. doi: 10.1016/S0006-3495(74)85947-3.

正常和强直性肌纤维中重构的动作电位。

Action potentials reconstructed in normal and myotonic muscle fibres.

作者信息

Adrian R H, Marshall M W

出版信息

J Physiol. 1976 Jun;258(1):125-43. doi: 10.1113/jphysiol.1976.sp011410.

DOI:10.1113/jphysiol.1976.sp011410
PMID:940049
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1308963/
Abstract
  1. Muscle fibres from goats with myotonia congenita show characteristic responses to stimulation with intracellular currents (Adrian & Bryant, 1974). To test whether the reduced surface chloride conductance can account for these myotonic discharges, we have calculated responses of a model 'muscle fibre' to intracellular current of long duration (greater than 100 msec), assuming that the current is applied at the end of the fibre, that the fibre is of finite length, that a regenerative action potential occurs in the transverse tubular system as well as the surface, and that the potassium current in the wall of the transverse tubular system raises the potassium in the tubular lumen. In the absence of information about the kinetic parameters of the ionic currents in mammalian muscle we have used numerical values from frog muscle (Adrian, Chandler & Hodgkin, 1970). 2. In calculations with a normal surface chloride conductance a long maintained current gives only one action potential. Reduction of the chloride conductance to a half produces repetitive firing during the current; reduction to a tenth produces repetitive firing during and a small number of action potentials after the end of the current. Elimination of the tubular potassium accumulation from the calculation reduces the number but does not eliminate action potentials arising after the end of the applied current. 3. With a tenth of the normal chloride conductance calculated responses show maintained firing following a constant current if the deactivating rate of the sodium channels (betam) is reduced by 25%. As before, eliminating potassium accumulation reduces the number of post-stimulus action potentials, but it does not eliminate them altogether. 4. We conclude that in the absence of a surface chloride conductance tubular potassium accumulation could certainly contribute to the instability of the membrane, but it is clear that potassium accumulation is not the only reason for the instability of myotonic muscle fibres. The kinetics of the sodium channels are important and we do not know that they are the same in normal and myotonic fibres. Nevertheless the presence of a surface chloride conductance does stabilize the response of a fibre to constant current or to repetitive stimulation, and its absence could be a sufficient condition for myotonic behaviour.
摘要
  1. 患有先天性肌强直的山羊的肌纤维对细胞内电流刺激呈现出特征性反应(阿德里安和布赖恩特,1974年)。为了测试表面氯化物电导降低是否能解释这些肌强直放电现象,我们计算了一个模型“肌纤维”对长时间(大于100毫秒)细胞内电流的反应,假设电流施加在纤维末端,纤维长度有限,在横管系统以及表面会产生再生性动作电位,并且横管系统壁中的钾电流会使管腔内的钾升高。由于缺乏关于哺乳动物肌肉中离子电流动力学参数的信息,我们使用了来自青蛙肌肉的数值(阿德里安、钱德勒和霍奇金,1970年)。2. 在具有正常表面氯化物电导的计算中,长时间持续的电流只会产生一个动作电位。将氯化物电导降低到一半会在电流期间产生重复放电;降低到十分之一会在电流期间产生重复放电,并在电流结束后产生少量动作电位。从计算中消除管内钾积累会减少动作电位的数量,但不会消除施加电流结束后产生的动作电位。 3. 当氯化物电导为正常的十分之一时,如果钠通道的失活速率(βm)降低25%,计算得到的反应显示在恒定电流后会持续放电。和之前一样,消除钾积累会减少刺激后动作电位的数量,但不会完全消除它们。4. 我们得出结论,在没有表面氯化物电导的情况下,管内钾积累肯定会导致膜的不稳定性,但很明显钾积累不是肌强直肌纤维不稳定性的唯一原因。钠通道的动力学很重要,而且我们不知道它们在正常纤维和肌强直纤维中是否相同。然而,表面氯化物电导的存在确实会使纤维对恒定电流或重复刺激的反应稳定,而其缺失可能是肌强直行为的充分条件