GRXCR2 Regulates Taperin Localization Critical for Stereocilia Morphology and Hearing

Publication date: 30 October 2018Source: Cell Reports, Volume 25, Issue 5Author(s): Chang Liu, Na Luo, Chun-Yu Tung, Benjamin J. Perrin, Bo ZhaoSummaryMutations in human GRXCR2, which encodes a protein of undetermined function, cause hearing loss by unknown mechanisms. We found that mouse GRXCR2 localizes to the base of the stereocilia, which are actin-based mechanosensing organelles in cochlear hair cells that convert sound-induced vibrations into electrical signals. The stereocilia base also contains taperin, another protein of unknown function required for human hearing. We show that taperin and GRXCR2 form a complex and that taperin is diffused throughout the stereocilia length in Grxcr2-deficient hair cells. Stereocilia lacking GRXCR2 are longer than normal and disorganized due to t...

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