The structural study aim to elucidate congenital hearing loss caused by OTOF gene mutation

Research Project

Project/Area Number	22791641
Research Category	Grant-in-Aid for Young Scientists (B)
Allocation Type	Single-year Grants
Research Field	Otorhinolaryngology
Research Institution	独立行政法人国立病院機構東京医療センター(臨床研究センター) (2011) 独立行政法人国立病院機構(東京医療センター臨床研究センター) (2010)
Principal Investigator	NAMBA Kazunori 独立行政法人国立病院機構東京医療センター(臨床研究センター), 臨床研究センター, 研究員 (60425684)
Project Period (FY)	2010 – 2011
Project Status	Completed (Fiscal Year 2011)
Budget Amount *help	¥3,770,000 (Direct Cost: ¥2,900,000、Indirect Cost: ¥870,000) Fiscal Year 2011: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000) Fiscal Year 2010: ¥2,730,000 (Direct Cost: ¥2,100,000、Indirect Cost: ¥630,000)
Keywords	耳科学 / 遺伝子変異 / OTOF / Auditory Neuropathy / 形態 / 難聴 / 蛋白質 / 蝸牛神経 / 内耳 / 立体構造
Research Abstract	In this study, a hypothetical phosphate signaling which is predicted from molecular modeling of Erk1-like structure of Otoferlin was explored using cultured cells and OTOF gene deficient mouse. In morphological investigation of spiral ganglion neuron of OTOF gene deficient mouse cochlea, a novel hypoganglionosis like phenotype was detected. The phenotype has a potential for the first morphological model of Auditory Neuropathy.

Report

(3 results)