Verselis Vytas K
Dominick P. Purpura Department of Neuroscience, Albert Einstein College of Medicine, Bronx, NY 10461, United States.
Neurosci Lett. 2019 Mar 16;695:40-45. doi: 10.1016/j.neulet.2017.09.020. Epub 2017 Sep 14.
Connexins play vital roles in hearing, including promoting cochlear development and sustaining auditory function in the mature cochlea. Mutations in connexins expressed in the cochlear epithelium, Cx26 and Cx30, cause sensorineural deafness and in the case of Cx26, is one of the most common causes of non-syndromic, hereditary deafness. Connexins function as gap junction channels and as hemichannels, which mediate intercellular and transmembrane signaling, respectively. Both channel configurations can play important, but very different roles in the cochlea. The potential roles connexin hemichannels can play are discussed both in normal cochlear function and in promoting pathogenesis that can lead to hearing loss.
连接蛋白在听力中发挥着至关重要的作用,包括促进耳蜗发育以及维持成熟耳蜗的听觉功能。在耳蜗上皮中表达的连接蛋白Cx26和Cx30发生突变会导致感音神经性耳聋,就Cx26而言,它是导致非综合征性遗传性耳聋的最常见原因之一。连接蛋白作为间隙连接通道和半通道发挥作用,分别介导细胞间和跨膜信号传导。这两种通道结构在耳蜗中都能发挥重要但截然不同的作用。本文将探讨连接蛋白半通道在正常耳蜗功能以及促进导致听力损失的发病机制中可能发挥的作用。