Transgenic Tmc2 expression preserves inner ear hair cells and vestibular function in mice lacking Tmc1

Yukako Asai, Bifeng Pan, Carl Nist-Lund, Alice Galvin, Andrei N. Lukashkin, Victoria A. Lukashkina, Tianwen Chen, Wu Zhou, Hong Zhu, Ian J. Russell, Jeffrey R. Holt, Gwenaelle S.G. Géléoc

    Research output: Contribution to journalArticlepeer-review


    Recent work has demonstrated that transmembrane channel-like 1 protein (TMC1) is an essential component of the sensory transduction complex in hair cells of the inner ear. A closely related homolog, TMC2, is expressed transiently in the neonatal mouse cochlea and can enable sensory transduction in Tmc1-null mice during the first postnatal week. Both TMC1 and TMC2 are expressed at adult stages in mouse vestibular hair cells. The extent to which TMC1 and TMC2 can substitute for each other is unknown. Several biophysical differences between TMC1 and TMC2 suggest these proteins perform similar but not identical functions. To investigate these differences, and whether TMC2 can substitute for TMC1 in mature hair cells, we generated a knock-in mouse model allowing Cre-inducible expression of Tmc2. We assayed for changes in hair cell sensory transduction and auditory and vestibular function in Tmc2 knockin mice (Tm[Tmc2]) in the presence or absence of endogenous Tmc1, Tmc2 or both. Our results show that expression of Tm[TMC2] restores sensory transduction in vestibular hair cells and transiently in cochlear hair cells in the absence of TMC1. The cellular rescue leads to recovery of balance but not auditory function. We conclude that TMC1 provides some additional necessary function, not provided by TMC2.

    Original languageEnglish
    Article number12124
    Pages (from-to)1-15
    Number of pages15
    JournalScientific Reports
    Issue number1
    Publication statusPublished - 14 Aug 2018

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