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Functional Analysis of Tmie in the Inner Ear and Gene Therapy using tmie Transgenic Mice
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- 발행기관
- 한국동물생명공학회(구 한국동물번식학회) 바로가기
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- 간행물
- 발생공학 국제심포지엄 및 학술대회 바로가기
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- 통권
- The 11th International Symposium on Developmental Biotechnology (2011.10)바로가기
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- 페이지
- pp.9-9
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- 저자
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- 언어
- 영어(ENG)
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- URL
- https://www.earticle.net/Article/A156540
원문정보
초록
- 영어
- The circling (cir/cir ) mouse is a murine model for human non‐syndromic deafness DFNB6. The causative gene is transmembrane inner ear (tmie), in which the mutation is a 40‐kilobase genomic deletion including tmie. The function of Tmie is unknown. To better understand the function of Tmie, we observed the spatiotemporal expression of tmie in the mouse cochlea using a Tmie‐specific antibody during postnatal inner ear development. Tmie was expressed in the cochlear hair cells of the mouse inner ear from embryonic days to adult. It is postulated that Tmie protein is involved in the hair cell structural formation and maturation before hearing onset (around P14), and maintenance of organ of Corti tissues after that. The cochlear hair cells of the circling mouse showed a basal‐to‐apical gradient of outer hair cell degeneration. The hair cell stereocilia bundles revealed the abnormal structure and it expanded to the apical region. In order to find the exact localization of Tmie protein inside the cell, we transfected the plasmids expressing GFP‐Tmie fusion protein into the HEI‐OC1 auditory hair cells. Tmie protein was colocalized with Calnexin (Canx), ER marker protein, but not with beta‐COP, Golgi marker protein. We next produced the Myo7a promoterdirected tmie expression transgenic mice to induce the phenotypic rescue of circling mice in a gene therapeutic way. Some circling mice with tmie transgene showed the normal behavior and hearing ability. These results indicate that tmie has a critical role in the inner ear development and hearing ability in the mice.
저자
간행물 정보
발행기관
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- 발행기관명
- 한국동물생명공학회(구 한국동물번식학회) [The Korean Society of Animal Reproduction and Biotechnology]
- 설립연도
- 1976
- 분야
- 농수해양>축산학
- 소개
- 동물번식생리학, 동물생명공학, 수의학, 인공수정 및 수정란이식을 이용한 동물개량에 관한 이론과 기술의 발전을 통해 학계, 연구계, 산업계 및 양축가 상호간의 협력을 도모함으로써 동물과학발전 및 사회일반의 이익에 기여 한다는 목적을 위해 노력해 나가겠습니다.
간행물
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- 간행물명
- 발생공학 국제심포지엄 및 학술대회 [International Symposium on Developmental Biotechnology]
- 간기
- 연간
- 수록기간
- 2004~2018
- 십진분류
- KDC 527 DDC 636
이 권호 내 다른 논문 / 발생공학 국제심포지엄 및 학술대회 The 11th International Symposium on Developmental Biotechnology
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