Transmembrane channel-like (TMC) genes are required for auditory and vestibular mechanosensation.
Transmembrane channel-like (TMC) genes are required for auditory and vestibular mechanosensation.
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DOI：
10.1007/s00424-014-1582-3
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发表时间：
2015-01
影响因子：
4.5
通讯作者：
Holt, Jeffrey R.
中科院分区：
文献类型：
--
作者：
Kawashima, Yoshiyuki;Kurima, Kiyoto;Pan, Bifeng;Griffith, Andrew J.;Holt, Jeffrey R.
关键词：
跨膜通道样1（TMC 1）基因突变可导致人类和小鼠显性和隐性耳聋。TMC 1是哺乳动物TMC基因中功能未知的8个基因之一。它们编码的蛋白质的多通道跨膜拓扑结构表明其作为受体、转运蛋白、通道或泵的作用。Tmc 1和密切相关的Tmc 2基因在小鼠内耳的听觉和前庭终器的神经感觉毛细胞中表达。最近的研究表明，Tmc 1和Tmc 2是毛细胞中机械电转导所必需的。这些蛋白质在机械电转导中的确切作用是未知的。TMC1和TMC2是毛细胞机械电转导通道的可行候选者，其组成分子已经逃避了30多年的鉴定。我们期望TMC蛋白的研究将产生对听觉和前庭毛细胞以及其他组织和器官中机械感觉的分子组成和机制的见解。
Mutations of the transmembrane channel-like 1 (TMC1) gene can cause dominant and recessive forms of deafness in humans and mice. TMC1 is one of eight mammalian TMC genes of unknown function. The multi-pass transmembrane topologic structure of the proteins they encode suggests roles as a receptor, transporter, channel or pump. Tmc1 and the closely related Tmc2 gene are expressed in neurosensory hair cells of the auditory and vestibular end organs of the mouse inner ear. Recent studies have demonstrated that Tmc1 and Tmc2 are specifically required for mechanoelectrical transduction in hair cells. The exact role of these proteins in mechanoelectrical transduction is unknown. TMC1 and TMC2 are viable candidates for the mechanoelectrical transduction channel of hair cells, whose component molecules have eluded identification for over 30 years. We expect that studies of TMC proteins will yield insights into molecular components and mechanisms of mechanosensation in auditory and vestibular hair cells, as well as in other tissues and organs.
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