Ectopic expression of tmie transgene induces various recovery levels of behavior and hearing ability in the circling mouse

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dc.contributor.authorM J Shin-
dc.contributor.authorJ H Lee-
dc.contributor.authorD H Yu-
dc.contributor.authorB S Kim-
dc.contributor.authorH J Kim-
dc.contributor.authorS H Kim-
dc.contributor.authorM O Kim-
dc.contributor.authorC Park-
dc.contributor.authorByung Hwa Hyun-
dc.contributor.authorS Lee-
dc.contributor.authorH S So-
dc.contributor.authorR Park-
dc.contributor.authorZ Y Ryoo-
dc.date.accessioned2017-04-19T09:11:25Z-
dc.date.available2017-04-19T09:11:25Z-
dc.date.issued2008-
dc.identifier.issn0006-291X-
dc.identifier.uri10.1016/j.bbrc.2008.06.064ko
dc.identifier.urihttps://oak.kribb.re.kr/handle/201005/8544-
dc.description.abstractThe circling (cir/cir) mouse is one of the murine models for human non-syndromic deafness DFNB6. The mice have abnormal circling behavior, suggesting a balanced disorder and profound deafness. The causative gene was transmembrane inner ear (tmie) gene of which the mutation is a 40-kb genomic deletion including tmie gene itself. In this study, tmie-overexpression trasngenic mice were established. Individuals with germline transmission have been mated with circling homozygous mutant mice (cir/cir) in order to produce the transgenic mutant mice (cir/cir-tg) as a gene therapy. After the genotyping, phenotypic analyses were performed so that the insertion of the new gene might compensate for the diseases such as hearing loss, circling behavior, or swimming inability. Some individuals exhibited complete recovery in their behavior and hearing but the others did not show any amelioration in behavior or hearing. Individual mice had very different levels of tmie transgene expression in the cochlea. These results clearly indicate that tmie protein plays an important role when the appropriate expression level of tmie was expressed in the inner ear. The protein levels were variable in each individual and these are thought to induce the differences in disease amelioration levels.-
dc.publisherElsevier-
dc.titleEctopic expression of tmie transgene induces various recovery levels of behavior and hearing ability in the circling mouse-
dc.title.alternativeEctopic expression of tmie transgene induces various recovery levels of behavior and hearing ability in the circling mouse-
dc.typeArticle-
dc.citation.titleBiochemical and Biophysical Research Communications-
dc.citation.number1-
dc.citation.endPage21-
dc.citation.startPage17-
dc.citation.volume374-
dc.contributor.affiliatedAuthorByung Hwa Hyun-
dc.contributor.alternativeName신미정-
dc.contributor.alternativeName이정한-
dc.contributor.alternativeName유동훈-
dc.contributor.alternativeName김봉수-
dc.contributor.alternativeName김희정-
dc.contributor.alternativeName김성현-
dc.contributor.alternativeName김명옥-
dc.contributor.alternativeName박찬이-
dc.contributor.alternativeName현병화-
dc.contributor.alternativeName이상규-
dc.contributor.alternativeName소홍섭-
dc.contributor.alternativeName박내길-
dc.contributor.alternativeName유자영-
dc.identifier.bibliographicCitationBiochemical and Biophysical Research Communications, vol. 374, no. 1, pp. 17-21-
dc.identifier.doi10.1016/j.bbrc.2008.06.064-
dc.subject.keywordCircling mouse-
dc.subject.keywordHearing loss-
dc.subject.keywordOrgan of Corti-
dc.subject.keywordtmie-
dc.subject.keywordTransgenic animal-
dc.subject.localCircling mouse-
dc.subject.localhearing loss-
dc.subject.localHearing loss-
dc.subject.localOrgan of Corti-
dc.subject.localtmie-
dc.subject.localtransgenic animal-
dc.subject.localTransgenic animals-
dc.subject.localTransgenic animal-
dc.description.journalClassY-
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