Jentsch T J, Günther W, Pusch M, Schwappach B
Centre for Molecular Neurobiology Hamburg (ZMNH), Hamburg University, Germany.
J Physiol. 1995 Jan;482(P):19S-25S. doi: 10.1113/jphysiol.1995.sp020560.
We review the properties of ClC chloride channels, members of an expanding gene family originally discovered by the cloning of the ClC-0 chloride channel from Torpedo electric organ. There are at least nine different ClC genes in mammals, several of which seem to be expressed ubiquitously, while others are expressed in a highly specific manner (e.g. the muscle-specific ClC-1 channel and the kidney-specific ClC-K channels). The newly cloned rat ClC-4 is strongly expressed in liver and brain, but also in heart, muscle, kidney and spleen. ClC chloride channels are structurally unrelated to other channel proteins and have twelve putative transmembrane domains. They function as multimers with probably four subunits. Functional characterization is most advanced with ClC-0, ClC-1 (mutations which cause myotonia) and ClC-2, a swelling-activated chloride channel. Many of the new ClC family members cannot yet be expressed functionally.
我们回顾了ClC氯离子通道的特性，它是一个不断扩展的基因家族的成员，最初是通过克隆来自电鳐电器官的ClC-0氯离子通道而发现的。哺乳动物中至少有9种不同的ClC基因，其中几种似乎在全身广泛表达，而其他一些则以高度特异性的方式表达（例如肌肉特异性的ClC-1通道和肾脏特异性的ClC-K通道）。新克隆的大鼠ClC-4在肝脏和大脑中强烈表达，但在心脏、肌肉、肾脏和脾脏中也有表达。ClC氯离子通道在结构上与其他通道蛋白无关，有12个假定的跨膜结构域。它们以可能由四个亚基组成的多聚体形式发挥作用。对ClC-0、ClC-1（导致肌强直的突变）和ClC-2（一种肿胀激活的氯离子通道）的功能特性研究最为深入。许多新的ClC家族成员尚未能实现功能性表达。
