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CIB2 interacts with TMC1 and TMC2 and is essential for mechanotransduction in auditory hair cells

Author

Listed:
  • Arnaud P. J. Giese

    (University of Maryland)

  • Yi-Quan Tang

    (Cambridge Biomedical Campus)

  • Ghanshyam P. Sinha

    (University of Kentucky)

  • Michael R. Bowl

    (MRC Harwell Institute, Mammalian Genetics Unit, Harwell Campus)

  • Adam C. Goldring

    (University of Wisconsin)

  • Andrew Parker

    (MRC Harwell Institute, Mammalian Genetics Unit, Harwell Campus)

  • Mary J. Freeman

    (University of Kentucky)

  • Steve D. M. Brown

    (MRC Harwell Institute, Mammalian Genetics Unit, Harwell Campus)

  • Saima Riazuddin

    (University of Maryland)

  • Robert Fettiplace

    (University of Wisconsin)

  • William R. Schafer

    (Cambridge Biomedical Campus)

  • Gregory I. Frolenkov

    (University of Kentucky)

  • Zubair M. Ahmed

    (University of Maryland)

Abstract

Inner ear hair cells detect sound through deflection of stereocilia, the microvilli-like projections that are arranged in rows of graded heights. Calcium and integrin-binding protein 2 is essential for hearing and localizes to stereocilia, but its exact function is unknown. Here, we have characterized two mutant mouse lines, one lacking calcium and integrin-binding protein 2 and one carrying a human deafness-related Cib2 mutation, and show that both are deaf and exhibit no mechanotransduction in auditory hair cells, despite the presence of tip links that gate the mechanotransducer channels. In addition, mechanotransducing shorter row stereocilia overgrow in hair cell bundles of both Cib2 mutants. Furthermore, we report that calcium and integrin-binding protein 2 binds to the components of the hair cell mechanotransduction complex, TMC1 and TMC2, and these interactions are disrupted by deafness-causing Cib2 mutations. We conclude that calcium and integrin-binding protein 2 is required for normal operation of the mechanotransducer channels and is involved in limiting the growth of transducing stereocilia.

Suggested Citation

  • Arnaud P. J. Giese & Yi-Quan Tang & Ghanshyam P. Sinha & Michael R. Bowl & Adam C. Goldring & Andrew Parker & Mary J. Freeman & Steve D. M. Brown & Saima Riazuddin & Robert Fettiplace & William R. Sch, 2017. "CIB2 interacts with TMC1 and TMC2 and is essential for mechanotransduction in auditory hair cells," Nature Communications, Nature, vol. 8(1), pages 1-13, December.
    • Handle: RePEc:nat:natcom:v:8:y:2017:i:1:d:10.1038_s41467-017-00061-1
    DOI: 10.1038/s41467-017-00061-1
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    Cited by:

    1. Martina Nicoletti & Letizia Chiodo & Alessandro Loppini, 2021. "Biophysics and Modeling of Mechanotransduction in Neurons: A Review," Mathematics, MDPI, vol. 9(4), pages 1-32, February.

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