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2015 Fiscal Year Final Research Report

Establishment of iPS cells from spinal and bulbar muscular atrophy and the research of polyglutamine diseases

Research Project

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Project/Area Number 25860722
Research Category

Grant-in-Aid for Young Scientists (B)

Allocation TypeMulti-year Fund
Research Field Neurology
Research InstitutionKeio University

Principal Investigator

Nihei Yoshihiro  慶應義塾大学, 医学部, 助教 (60468501)

Project Period (FY) 2013-04-01 – 2016-03-31
Keywordsポリグルタミン病 / 球脊髄性筋萎縮症 / 神経変性疾患 / 運動ニューロン疾患 / アンドロゲン受容体 / 17-AAG / iPS細胞
Outline of Final Research Achievements

We established spinal and bulbar muscular atrophy (SBMA)-derived iPS cells (iPSCs) and confirmed motor neuron differentiation. Aggregation of androgen receptor in SBMA-iPSC-derived neurons is enhanced by dihydrotestosterone. And one of the candidate drugs, 17-AAG promoted to degrade AR aggregation. These findings show iPSCs technology makes us be able to recapitulate disease-specific biochemical features and demonstrate the potential for identification and validation of candidate drugs. We also established iPSCs from other polyglutamine diseases (Machado-Joseph disease, Dentatorubral-pallidoluysian atrophy) and neurodegenerative diseases (amyotrophic lateral sclerosis, Parkinson's disease). We are going to advance the polyglutamine disease researches by using the established iPSC lines.

Free Research Field

神経変性疾患

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Published: 2017-05-10  

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