ClC型氯离子通道的研究

Research progress in ClC chloride channels

ClC型氯离子通道是一类分布广泛的阴离子通道,参与多种生理过程,如pH及静息膜电位和兴奋性的调节、细胞内囊泡的酸化和细胞体积调节等,其基因突变或功能异常都可导致多种疾病的发生。对于ClC型氯离子通道结构与功能关系的研究,有助于理解相关疾病的分子机制。本文简要介绍了ClC型氯离子通道的分类与性质、分子结构与作用机制、以及结构和表达异常所导致的主要生理变化和疾病。

Chloride is the most abundant extracellular anion in multicellular organisms.The voltage-gated ClC family of chloride channels is widely distributed in prokaryotic and eukaryotic cells and performs diverse physiological roles,including control of cellular excitability,acidification of intracellular vesicles,and regulation of cell volume.Mutations of ClC channels have been associated with human diseases.Clearly understanding of the structural -functional relationships of these proteins is critical in defining the molecular mechanisms underlying disease pathogenesis.In this article,literatures have been reviewed in terms of classification,structure,mechanism of ClC chloride channels and relevant physiological changes and diseases.