Analysis of retinal degeneration caused by dysfunction of ubiquitin proteasome system

2015 Fiscal Year Final Research Report

• Project/Area Number: 25462702
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Ophthalmology
• Research Institution: Hokkaido University
• Principal Investigator: NODA Mika 北海道大学, 医学(系)研究科(研究院), 客員研究員 (10296668)
• Co-Investigator(Kenkyū-buntansha): NODA Kousuke 北海道大学, 大学院医学研究科, 准教授 (90296666) ; TOMARU Utano 北海道大学, 大学院医学研究科, 准教授 (20360901) ; OZAWA Yoko 慶應義塾大学, 医学部(信濃町), 講師 (90265885)
• Project Period (FY): 2013-04-01 – 2016-03-31
• Keywords: プロテアソーム / 網膜色素変性

Outline of Final Research Achievements

Recent studies have revealed that ubiquitin proteasome system (UPS) dysfunction is involved in the pathogenesis of systemic neurodegenerative diseases such as Alzheimer’s disease. In ocular diseases, retinitis pigmentosa (RP) is a heterogeneous group of inherited retinal neurodegenerative disorders characterized by progressive degeneration of photoreceptors. Therefore, whether impairment of UPS function also causes RP has been of great interest.
In the present study, using an animal model of decreased proteasomal activity (β5t-Tg mice) we demonstrated that i) decreased function of proteasome causes retinal degeneration in vivo, largely attributed to photoreceptor cell death, and ii) photoreceptor cells are vulnerable to the functional disturbance of proteasome in the retina. The current data raise an interesting question regarding the impact of proteasome dysfunction in the pathogenesis of RP.

Free Research Field

眼科