Correction of Deafness in shaker-2 Mice by an Unconventional Myosin in a BAC Transgene

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Science  29 May 1998:
Vol. 280, Issue 5368, pp. 1444-1447
DOI: 10.1126/science.280.5368.1444

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The shaker-2 mouse mutation, the homolog of humanDFNB3, causes deafness and circling behavior. A bacterial artificial chromosome (BAC) transgene from the shaker-2critical region corrected the vestibular defects, deafness, and inner ear morphology of shaker-2 mice. An unconventional myosin gene, Myo15, was discovered by DNA sequencing of this BAC.Shaker-2 mice were found to have an amino acid substitution at a highly conserved position within the motor domain of this myosin. Auditory hair cells of shaker-2 mice have very short stereocilia and a long actin-containing protrusion extending from their basal end. This histopathology suggests that Myo15 is necessary for actin organization in the hair cells of the cochlea.

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