Pathological relation between retinal degeneration and modulation of transthyretin expression by amyloid precursor protein

Research Project

Project/Area Number	23700427
Research Category	Grant-in-Aid for Young Scientists (B)
Allocation Type	Multi-year Fund
Research Field	Nerve anatomy/Neuropathology
Research Institution	Jikei University School of Medicine
Principal Investigator	FUJIGASAKI Junko 東京慈恵会医科大学, 医学部, 講師 (60312021)
Project Period (FY)	2011-04-28 – 2015-03-31
Project Status	Completed (Fiscal Year 2014)
Budget Amount *help	¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000) Fiscal Year 2013: ¥650,000 (Direct Cost: ¥500,000、Indirect Cost: ¥150,000) Fiscal Year 2012: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000) Fiscal Year 2011: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
Keywords	脊髄小脳失調症 / アルツハイマー病 / 網膜変性 / 網膜色素上皮細胞
Outline of Final Research Achievements	Spinocerebellar ataxia 7 (SCA7) is an autosomal dominant neurodegenerative disorder characterized by cerebellar ataxia and retinal degeneration. SCA7, Alzheimer’s disease model and normal control mice were examined to elucidate mechanisms of retinal degeneration. In the photoreceptor and ganglion cells of SCA7, nuclear spliceosome altered its structure; these indicated that alteration of RNA metabolism might relate to retinal pathology of SCA7. Aggregation of amyloid precursor related proteins was observed in Alzheimer’s disease model mice. These novel findings will contribute to analyze pathogenesis of retinal degeneration in neurodegenerative disorders.