doi: 10.1126/science.280.5368.1444.
Correction of deafness in shaker-2 mice by an unconventional myosin in a BAC transgene
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- PMID: 9603735
- DOI: 10.1126/science.280.5368.1444
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Correction of deafness in shaker-2 mice by an unconventional myosin in a BAC transgene
Science.
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Abstract
The shaker-2 mouse mutation, the homolog of human DFNB3, causes deafness and circling behavior. A bacterial artificial chromosome (BAC) transgene from the shaker-2 critical 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.
Comment in
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More deafness genes.Science. 1998 May 29;280(5368):1403. doi: 10.1126/science.280.5368.1403. Science. 1998. PMID: 9634418 No abstract available.
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