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

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. Schafer, Gregory I. Frolenkov () and Zubair M. Ahmed ()
Additional contact information
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

Nature Communications, 2017, vol. 8, issue 1, 1-13

Abstract: 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.

Date: 2017
References: Add references at CitEc
Citations: View citations in EconPapers (1)

Downloads: (external link)
https://www.nature.com/articles/s41467-017-00061-1 Abstract (text/html)

Related works:
This item may be available elsewhere in EconPapers: Search for items with the same title.

Export reference: BibTeX RIS (EndNote, ProCite, RefMan) HTML/Text

Persistent link: https://EconPapers.repec.org/RePEc:nat:natcom:v:8:y:2017:i:1:d:10.1038_s41467-017-00061-1

Ordering information: This journal article can be ordered from
https://www.nature.com/ncomms/

DOI: 10.1038/s41467-017-00061-1

Access Statistics for this article

Nature Communications is currently edited by Nathalie Le Bot, Enda Bergin and Fiona Gillespie

More articles in Nature Communications from Nature
Bibliographic data for series maintained by Sonal Shukla () and Springer Nature Abstracting and Indexing ().