Division of Glia Disease and Therapeutics, Center for Translational Neuromedicine, University of Rochester Medical School, Rochester, NY 14640, USA.
Brain Res. 2012 Dec 3;1487:61-8. doi: 10.1016/j.brainres.2012.07.003. Epub 2012 Jul 10.
Several demyelinating syndromes have been linked to mutations in glial gap junction proteins, the connexins. Although mutations in connexins of the myelinating cells, Schwann cells and oligodendrocytes, were initially described, recent data have shown that astrocytes also play a major role in the demyelination process. Alterations in astrocytic proteins directly affect the oligodendrocytes' ability to maintain myelin structure, and associated astrocytic proteins that regulate water and ionic fluxes, including aquaporins, can also regulate myelin integrity. Here, we will review the main evidence from human disorders and transgenic mouse models that implicate glial gap junction proteins in demyelinating diseases and the therapeutic potential of some of these targets. This article is part of a Special Issue entitled Electrical Synapses.
几种脱髓鞘综合征与神经胶质细胞间隙连接蛋白（连接蛋白）的突变有关。尽管最初描述了髓鞘形成细胞（施万细胞和少突胶质细胞）的连接蛋白突变，但最近的数据表明，星形胶质细胞在脱髓鞘过程中也起着主要作用。星形胶质细胞蛋白的改变直接影响少突胶质细胞维持髓鞘结构的能力，并且调节水和离子通量的相关星形胶质细胞蛋白，包括水通道蛋白，也可以调节髓鞘的完整性。在这里，我们将回顾主要的人类疾病和转基因小鼠模型的证据，这些证据表明神经胶质细胞间隙连接蛋白与脱髓鞘疾病有关，以及其中一些靶点的治疗潜力。本文是特刊“电突触”的一部分。
