PATHOPHYSIOLOGY OF MYOTONIA PRODUCED BY AROMATIC CARBOXYLIC-ACIDS
PATHOPHYSIOLOGY OF MYOTONIA PRODUCED BY AROMATIC CARBOXYLIC-ACIDS
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DOI：
10.1002/ana.410040411
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发表时间：
1978-01-01
影响因子：
11.2
通讯作者：
BARCHI, RL
中科院分区：
文献类型：
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作者：
FURMAN, RE;BARCHI, RL
一系列的9个相关的芳香族一元羧酸（ACA）先前显示抑制肌肉膜氯离子电导（GCl）选择性在大鼠进行了研究，他们的能力，以产生肌强直。所有九个诱导特征重复的电活动和延迟放松在孤立的肌肉，虽然这种行动所需的浓度变化很大。在每种情况下，在与先前测定的GCl抑制半最大浓度密切相关的浓度下观察到肌强直。从用ACA进行肌强直的肌肉的细胞内记录显示，在基强度处的延长的潜伏期、多个驱动尖峰和自我维持的重复活动与先前在遗传性山羊肌强直中报道的相似。暴露于这些化合物中最有效的化合物后记录的膜动作电位的相平面图表明，对电压依赖性钠系统的影响很小。所看到的变化可以通过简单的去除氯离子来复制。在低膜GCl存在下的重复电活动的表达依赖于环境温度和钙离子浓度。升高温度和降低Ca++倾向于肌强直活动;匡威的条件抑制肌强直。ACA诱导的肌强直可被临床上有效控制人类遗传性肌强直的浓度的二苯乙内酰脲抑制。
A series of nine related aromatic monocarboxylic acids (ACAs) previously shown to inhibit muscle membrane chloride conductance (GCl) selectively in the rat were studied for their ability to produce myotonia. All nine induced characteristic repetitive electrical activity and delayed relaxation in isolated muscle, although the concentrations required for this action varied widely. In each case, myotonia was observed at concentrations that correlated closely with previously determined half‐maximal concentrations for inhibition of GCl. Intracellular recordings from muscle made myotonic with ACA revealed prolonged latencies at rheobase, multiple driven spikes, and self‐sustaining repetitive activity similar to that previously reported in hereditary goat myotonia. Phase‐plane diagrams of membrane action potentials recorded after exposure to the most effective of these compounds suggested little effect on the voltage‐dependent sodium system. The changes seen could be duplicated by simple removal of chloride ion.The expression of repetitive electrical activity in the presence of low membrane GCldepends on ambient temperature and on the concentration of calcium ion. Increasing temperature and decreasing Ca++predispose toward myotonic activity; converse conditions inhibit myotonia. Myotonia induced by ACA is inhibited by concentrations of diphenylhydantoin that are clinically effective in controlling hereditary myotonia in humans.