CELL BIOLOGY: Whirlin to the Tip

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Science  28 Jan 2005:
Vol. 307, Issue 5709, pp. 485c
DOI: 10.1126/science.307.5709.485c

Hearing depends on the neat arrangement of stereocilia in graduated rows on the apical surface of hair cells in the inner ear. Disruption of this architecture interferes with the ability to detect both sounds and head movement. The architecture is established by the presence of actin cores within each stereocilium of a defined length. Two mutant mice strains possess abbreviated stereocilia—the shaker 2 and whirler mice. Shaker 2 mice are deficient in the production of a motor protein, myosin XVa, and whirler mice are deficient in a protein termed whirlin. Belyantseva et al. now show that the myosin XVa protein interacts with whirlin and promotes its delivery to the tips of stereocilia. When this interaction is disrupted, stereocilia are abnormal and deafness will ensue. The whirlin transport defect, and the aberrant hair bundle pattern, in hair cells taken from shaker 2 mice could be “cured” by transfection with a fluorescently tagged version of myosin XVa. — SMH

Nature Cell Biol. 10.1038/ncb1219 (2005).

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