Barchi R L
Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia 19104, USA.
Neurobiol Dis. 1997;4(3-4):254-64. doi: 10.1006/nbdi.1997.0158.
Voltage-gated ion channels play a critical role in coupling excitation at the neuromuscular junction to activation of contractile elements within a muscle fiber. Abnormal channel function can lead to either muscle paralysis or delayed relaxation. Recent advances in the molecular characterization of these ion channels have provided the tools needed to investigate the relationship between channel mutations and disorders of muscle excitability. This article reviews our current understanding of muscle sodium, calcium, and chloride channels and their role in the pathogenesis of myotonia and periodic paralysis.
电压门控离子通道在神经肌肉接头处的兴奋与肌纤维内收缩元件的激活之间的耦合中起着关键作用。通道功能异常可导致肌肉麻痹或延迟松弛。这些离子通道分子特征的最新进展提供了研究通道突变与肌肉兴奋性障碍之间关系所需的工具。本文综述了我们目前对肌肉钠、钙和氯通道及其在肌强直和周期性麻痹发病机制中的作用的理解。
