Correction of Deafness in shaker-2 Mice by an Unconventional Myosin in a BAC Transgene
- 29 May 1998
- journal article
- other
- Published by American Association for the Advancement of Science (AAAS) in Science
- Vol. 280 (5368) , 1444-1447
- https://doi.org/10.1126/science.280.5368.1444
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.Keywords
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