Centre of Excellence in Neuromics, CHUM Research Center and Department of Medicine, University of Montreal, Notre-Dame Hospital, 1560 Sherbrooke East, De-Seve Pavillion, room Y-3616-2, Montréal, QC, H2L 4M1, Canada.
Brain Res. 2011 Feb 16;1374:15-26. doi: 10.1016/j.brainres.2010.12.010. Epub 2010 Dec 11.
Potassium/Chloride cotransporters are transmembrane proteins that regulate cell volume and control neuronal activity by transporting K(+) and Cl(-) ions across the plasma membrane. Potassium/Chloride cotransporter 3 (KCC3) mutations are responsible for hereditary motor and sensory neuropathy with agenesis of the corpus callosum (HMSN/ACC), which is a severe sensory and motor neuropathy. Two major splice variants, KCC3a and KCC3b, were shown to be expressed in adult mouse tissues. Although KCC3a is mainly expressed in the central nervous system (CNS), its specific cellular expression patterns have not been determined. Here, we used an approach combining in situ hybridization and immunohistochemical techniques to determine the cellular expression of KCC3 in the mouse CNS and showed that KCC3 is mainly expressed in neurons, including a subpopulation of interneurons. Finally, we showed that some non-neuronal cells, such as radial glial-like cells in the spinal cord, also express KCC3.
钾/氯离子共转运蛋白是跨膜蛋白，通过在质膜上转运 K(+)和 Cl(-)离子来调节细胞体积并控制神经元活动。钾/氯离子共转运蛋白 3 (KCC3)突变负责遗传性运动感觉神经病伴胼胝体发育不全 (HMSN/ACC)，这是一种严重的感觉运动神经病。两种主要的剪接变体 KCC3a 和 KCC3b 被证明在成年小鼠组织中表达。尽管 KCC3a 主要在中枢神经系统 (CNS) 中表达，但它的特定细胞表达模式尚未确定。在这里，我们使用结合原位杂交和免疫组织化学技术的方法来确定 KCC3 在小鼠中枢神经系统中的细胞表达，并表明 KCC3 主要表达在神经元中，包括中间神经元的一个亚群。最后，我们表明一些非神经元细胞，如脊髓中的放射状胶质样细胞，也表达 KCC3。
