XIRP2, an actin-binding protein essential for inner ear hair-cell stereocilia

Déborah I. Scheffer, Duan Sun Zhang, Jun Shen, Artur Indzhykulian, K. Domenica Karavitaki, Yichao Joy Xu, Qinchuan Wang, Jim Jung Ching Lin, Zheng Yi Chen, David P. Corey

Research output: Contribution to journalArticlepeer-review

Abstract

Hair cells of the inner ear are mechanoreceptors for hearing and balance, and proteins highly enriched in hair cells may have specific roles in the development and maintenance of the mechanotransduction apparatus. We identified XIRP2/mXinβ as an enriched protein likely to be essential for hair cells. We found that different isoforms of this protein are expressed and differentially located: short splice forms (also called XEPLIN) are targeted more to stereocilia, whereas two long isoforms containing a XIN-repeat domain are in both stereocilia and cuticular plates. Mice lacking the Xirp2 gene developed normal stereocilia bundles, but these degenerated with time: stereocilia were lost and long membranousprotrusions emanated from the nearby apical surfaces. At an ultrastructural level, the paracrystalline actin filaments became disorganized. XIRP2 isapparently involved in the maintenance of actin structures in stereocilia and cuticular plates of haircells, and perhaps in other organs where it is expressed.

Original languageEnglish (US)
Pages (from-to)1811-1818
Number of pages8
JournalCell Reports
Volume10
Issue number11
DOIs
StatePublished - Mar 24 2015
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

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