Attempted rescue of circling mice by hair cell-specific expression (Myo7a promoter) of tmie transgene

Author:

Park Seojin,Jang Soyoung,Lee Jeong-Han,Kim Sung-Hyun,Ryoo Zae Young

Abstract

The spontaneous mutant circling mouse (cir/cir) is deaf and displays abnormal behavior, particularly circling and head tossing. To rescue the circling mouse phenotype, we produced transgenic mice with hair cell-specific expression of the transmembrane inner ear (tmie) gene, using the Myo7a promoter, and generated cir/cir homozygous mice carrying the transgene (cir/cir-tg<sup>Myo7a</sup>) by breeding with circling mice. The cir/cir-tg<sup>Myo7a</sup> mice still exhibited circling behavior and were unable to swim in water unlike cir/cir-tg<sup>CMV</sup> mice and wild-type mice. An auditory brainstem response (ABR) test demonstrated that the cir/cir-tgMyo7a mice could not respond to sound. Immunohistochemical analysis showed enhanced green fluorescent protein, a marker of tmie expression, in the inner and outer hair cells of the cir/cir-tg<sup>Myo7a</sup> mice. Hair cells and spiral ganglion neurons in the cir/cir-tg<sup>Myo7a</sup> mice were recovered, but not completely. This study demonstrates that tmie transgene expression in hair cells alone could not restore wild-type hearing and behavior in circling mice.

Publisher

Medycyna Weterynaryjna - Redakcja

Subject

General Veterinary

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