Disorders associated with Ion Channels

N. Johnkennedy
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Abstract

Muscular wasting, a lack of muscle tone, or sporadic muscle paralysis are the most common symptoms of ion channel diseases, which are linked to defects in the proteins known as ion channels. Ion channels are diverse and differ with respect to how they open and close (gating) and their ionic conductance and selectivity (permeation). The development of novel molecules to modulate their activity, fundamental understanding of ion channel structure-function mechanisms, their physiological functions, how their dysfunction results in disease, their use as biosensors, and their utility as biosensors are important and active research frontiers. With a particular emphasis on voltage-gated ion channels, ion-channel engineering methods that have been used to investigate these facets of ion channel function are x-rayed.
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与离子通道相关的疾病
肌肉萎缩、缺乏肌肉张力或散发性肌肉麻痹是离子通道疾病最常见的症状,这与称为离子通道的蛋白质缺陷有关。离子通道是多种多样的,在它们如何打开和关闭(门控)以及它们的离子电导率和选择性(渗透)方面有所不同。发展新的分子来调节离子通道的活性,对离子通道结构-功能机制的基本理解,它们的生理功能,它们的功能障碍如何导致疾病,它们作为生物传感器的使用,以及它们作为生物传感器的用途是重要和活跃的研究前沿。特别强调电压门控离子通道,离子通道工程方法已用于研究离子通道功能的这些方面是x射线。
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