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2017 | 73 | 07 |

Tytuł artykułu

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

Warianty tytułu

Języki publikacji

EN

Abstrakty

EN
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.

Słowa kluczowe

Wydawca

-

Rocznik

Tom

73

Numer

07

Opis fizyczny

p.399-403,fig.,ref.

Twórcy

autor
autor
autor
  • Department of Physiology, School of Medicine, University of Nevada, Reno, Nevada, USA
autor
  • Center for Laboratory Animal Resources, Kyungpook National University, Daegu, Republic of Korea
autor
  • School of Life Sciences, BK21 Plus KNU creative bioresearch group, Kyungpook National University, Daegu, Republic of Korea

Bibliografia

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  • Cho K. I., Lee J. W., Kim K. S., Lee E. J., Suh J. G., Lee H. J., Kim H. T., Hong S. H., Chung W. H., Chang K. T., Hyun B. H., Oh Y. S., Ryoo Z. Y.: Fine mapping of the circling (cir) gene on the distal portion of mouse chromosome 9. Comp. Med. 2003, 53, 642-648.
  • Chung W. H., Kim K. R., Cho Y. S., Cho D. Y., Woo J. H., Ryoo Z. Y., Cho K. I., Hong S. H.: Cochlear pathology of the circling mouse: a new mouse model of DFNB6. Acta Oto-laryngol. 2007, 127, 244-251.
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  • Groves A. K., Zhang K. D., Fekete D. M.: The genetics of hair cell development and regeneration. Ann. Rev. Neurosci. 2013, 36, 361-381.
  • Hakizimana P., Brownell W. E., Jacob S., Fridberger A.: Sound-induced length changes in outer hair cell stereocilia. Nature Comm. 2012, 3, 1094.
  • Johnson S. L., Kuhn S., Franz C., Ingham N., Furness D. N., Knipper M., Steel K. P., Adelman J. P., Holley M. C., Marcotti W.: Presynaptic maturation in auditory hair cells requires a critical period of sensory-independent spiking activity. Proc. Nat. Acad. Sci. USA 2013, 110, 8720-8725.
  • Kwon T. J., Cho H. J., Kim U. K., Lee E., Oh S. K., Bok J., Bae Y. C., Yi J. K., Lee J. W., Ryoo Z. Y., Lee S. H., Lee K. Y., Kim H. Y.: Methionine sulfoxide reductase B3 deficiency causes hearing loss due to stereocilia degeneration and apoptotic cell death in cochlear hair cells. Hum. Mol. Gen. 2014, 23, 1591-1601.
  • Lee J. W., Lee E. J., Hong S. H., Chung W. H., Lee H. T., Lee T. W., Lee J. R., Kim H. T., Suh J. G., Kim T. Y., Ryoo Z. Y.: Circling mouse: possible animal model for deafness. Comp. Med. 2001, 51, 550-554.
  • Lee J. W., Ryoo Z. Y., Lee E. J., Hong S. H., Chung W. H., Lee H. T., Chung K. S., Kim T. Y., Oh Y. S., Suh J. G.: Circling mouse, a spontaneous mutant in the inner ear. Experimental Animals 2002, 51, 167-171.
  • Li S., Price S. M., Cahill H., Ryugo D. K., Shen M. M., Xiang M.: Hearing loss caused by progressive degeneration of cochlear hair cells in mice deficient for the Barhl1 homeobox gene. Development 2002, 129, 3523-3532.
  • Liu H., Pecka J. L., Zhang Q., Soukup G. A., Beisel K. W., He D. Z.: Characterization of transcriptomes of cochlear inner and outer hair cells. J. Neurosci. 2014, 34, 11085-11095.
  • Naz S., Giguere C. M., Kohrman D. C., Mitchem K. L., Riazuddin S., Morell R. J., Ramesh A., Srisailpathy S., Deshmukh D., Riazuddin S., Griffith A. J., Friedman T. B., Smith R. J., Wilcox E. R.: Mutations in a novel gene, TMIE, are associated with hearing loss linked to the DFNB6 locus. Am. Human Gen. 2002, 71, 632-636.
  • Park S., Lee J. H., Cho H. J., Lee K. Y., Kim M. O., Yun B. W., Ryoo Z.: tmie is required for gentamicin uptake by the hair cells of mice. Comp. Med. 2013, 63, 136-142.
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  • Shin M. J., Lee J. H., Yu D. H., Kim B. S., Kim H. J., Kim S. H., Kim M. O., Park C., Hyun B. H., Lee S., So H. S., Park R., Ryoo Z. Y.: Ectopic expression of tmie transgene induces various recovery levels of behavior and hearing ability in the circling mouse. Biochem. Biophys. Res. Comm. 2008, 374, 17-21.
  • Shin M. J., Lee J. H., Yu D. H., Kim H. J., Bae K. B., Yuh H. S., Kim M. O., Hyun B. H., Lee S., Park R., Ryoo Z. Y.: Spatiotemporal expression of tmie in the inner ear of rats during postnatal development. Comp. Med. 2010, 60, 288-294.
  • Zhao B., Wu Z., Grillet N., Yan L., Xiong W., Harkins-Perry S., Muller U.: TMIE is an essential component of the mechanotransduction machinery of cochlear hair cells. Neuron. 2014, 84, 954-967.
  • Zilberstein Y., Liberman M. C., Corfas G.: Inner hair cells are not required for survival of spiral ganglion neurons in the adult cochlea. J. Neurosci. 2012, 32, 405-410.

Typ dokumentu

Bibliografia

Identyfikatory

Identyfikator YADDA

bwmeta1.element.agro-d04904db-6ee5-4301-8e8a-71c1d9e8e905
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