Morphological analysis and gene therapy targeted the vestibular organ with Connexin 26 related hereditary deafness

2016 Fiscal Year Final Research Report

• Project/Area Number: 26462567
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Otorhinolaryngology
• Research Institution: Juntendo University
• Principal Investigator: OKADA Hiroko 順天堂大学, 医学部, 助教 (20433774)
• Project Period (FY): 2014-04-01 – 2017-03-31
• Keywords: 遺伝性難聴 / GJB2 / コネキシン26 / 前庭 / トランスジェニックマウス / 遺伝子治療 / アデノ随伴ウィルス

Outline of Final Research Achievements

We analyzed the morphological and functional development of the vestibular organ in Cx26 transgenic mice between 0 days after birth (P0) and P140, which was compared with that of littermate control mice (non-Tg). The gross structure of the inner ear in non-transgenic and transgenic mice revealed no hydrops, no defects, no degeneration in either the cochlea or the vestibule throughout the postnatal period. Light microscopic observations in the sensory epithelium revealed normally developed and matured utricle macula, saccular macula, and ampulla in the transgenic mice, which were very similar to those of the nontransgenic mouse. The present study clearly demonstrated that postnatal development and maturation in the vestibular organ were morphologically and functionally completed in Cx26 transgenic mice. Furthermore, we examined the gene transfer with adeno associated virus (AAV) via semicircular canal and successfully delivered AAV into the vestibular cells.

Free Research Field

耳鼻咽喉科学