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

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Authors

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

Issue Date

2017

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Article

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Keywords

circling mouse , hair cell , Myo7a promoter , tmie

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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-tgMyo7a) by breeding with circling mice. The cir/cir-tgMyo7a mice still exhibited circling behavior and were unable to swim in water unlike cir/cir-tgCMV 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-tgMyo7a mice. Hair cells and spiral ganglion neurons in the cir/cir-tgMyo7a 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.

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Park, S., Jang, S., Lee, J.-H., Kim, S.-H., & Ryoo, Z. Y. (2017). Attempted rescue of circling mice by hair cell-specific expression (Myo7a promoter) of tmie transgene. Medycyna Weterynaryjna, 73(7), 399–403. doi:10.21521/mw.5744

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ISSN

0025-8628

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