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首页> 外文期刊>Proceedings of the National Academy of Sciences of the United States of America >Properties of human connexin 31, which is implicated in hereditary dermatological disease and deafness
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Properties of human connexin 31, which is implicated in hereditary dermatological disease and deafness

机译：人类连接蛋白31的特性，与遗传性皮肤病和耳聋有关

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摘要

The connexins are a family of at least 20 homologous proteins in humans that form aqueous channels connecting the interiors of coupled cells and mediating electrical and chemical communication. Mutations in the gene for human connexin 31 (hCx31) are associated with disorders of the skin and auditory system. Alterations in functional properties of Cx31 junctions are likely to play a role in these diseases; nonetheless, little is known about the properties of the wild-type channels. Here we show that hCx31 channels, like other connexin channels, are gated by voltage and close at low pH and when exposed to long-chain alkanols. Single-channel conductance of the fully open channel is approximate to 85 pS, and it is permeable to Lucifer yellow, Alexa Fluor(350), ethidium bromide, and DAPI which have valences of -2, -1, +1, and +2, respectively. In contrast to what has been reported for mouse Cx31, hCx31 appears to form functional heterotypic channels with all four connexins tested, Cx26, Cx30, Cx32, and Cx45. These findings provide an important first step in evaluating the pathogenesis of inherited human diseases associated with mutations in the gene for Cx31.
机译：连接蛋白是人类中至少20种同源蛋白质的家族，它们形成连接耦合细胞内部并介导电和化学通讯的水通道。人连接蛋白31（hCx31）基因的突变与皮肤和听觉系统疾病有关。 Cx31连接的功能特性的改变很可能在这些疾病中起作用。然而，对于野生型通道的性质知之甚少。在这里，我们显示与其他连接蛋白通道一样，hCx31通道也受电压门控，并在低pH值下以及暴露于长链烷醇时关闭。全开放通道的单通道电导率约为85 pS，并且可渗透价为-2，-1，+ 1和+2的路西法黄，Alexa Fluor（350），溴化乙锭和DAPI ， 分别。与针对小鼠Cx31的报道相反，hCx31似乎与所有四种连接蛋白Cx26，Cx30，Cx32和Cx45形成功能异型通道。这些发现为评估与Cx31基因突变相关的人类遗传病的发病机理提供了重要的第一步。

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