Dataset Information

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

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/mXin? 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 membranous protrusions emanated from the nearby apical surfaces. At an ultrastructural level, the paracrystalline actin filaments became disorganized. XIRP2 is apparently involved in the maintenance of actin structures in stereocilia and cuticular plates of hair cells, and perhaps in other organs where it is expressed.

SUBMITTER: Scheffer DI

PROVIDER: S-EPMC4376604 | biostudies-literature | 2015 Mar

REPOSITORIES: biostudies-literature

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

Scheffer Déborah I DI Zhang Duan-Sun DS Shen Jun J Indzhykulian Artur A Karavitaki K Domenica KD Xu Yichao Joy YJ Wang Qinchuan Q Lin Jim Jung-Ching JJ Chen Zheng-Yi ZY Corey David P DP

Cell reports 20150312 11

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 contain ...[more]

PMID: 25772365

Dataset Information

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

Publications

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

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