Clarification of pathological condition and therapeutic development of Xeroderma pigmentosum with model mice experiments

2015 Fiscal Year Final Research Report

• Project/Area Number: 26861380
• Research Category: Grant-in-Aid for Young Scientists (B)
• Allocation Type: Multi-year Fund
• Research Field: Otorhinolaryngology
• Research Institution: Kobe University
• Principal Investigator: KOJIMA Yasutaka 神戸大学, 医学(系)研究科(研究院), 研究員 (80464270)
• Co-Investigator(Kenkyū-buntansha): YAMASHITA Daisuke 神戸大学, 医学部附属病院, 准教授 (60306785) ; FUJITA Takeshi 神戸大学, 医学研究科, 医学研究員 (90533711) ; SHINOMIYA Hitomi 神戸大学, 医学部附属病院, その他 (70623081)
• Co-Investigator(Renkei-kenkyūsha): NISHIGORI Chikako 神戸大学, 医学(系)研究科(研究院), 教授 (50198454)
• Project Period (FY): 2014-04-01 – 2016-03-31
• Keywords: 色素性乾皮症 / 感音難聴 / XPAモデルマウス

Outline of Final Research Achievements

For a treatment strategy for progressive sensorineural hearing loss in Xeroderma pigmentosum (XP), we evaluated a vulnerability of inner ears in XP complementation group A (XPA) model mice morphologically and physiologically. In auditory brainstem response, a hearing threshold of XPA model mice elevated higher than that of control group. In microscopic observation of sacrificed cochlear section, ganglion spiral cochlear cells decreased in XPA model mice. This is a first report on fragile inner ear in XPA model mice, therefore it becomes a cornerstone of inquisition of pathological condition and treatments of progressive sensorineural hearing loss in XP in the future.

Free Research Field

耳鼻咽喉科