In vitro "virtual biopsy" of cochlear cells by using Wolfram syndrome patients derived hiPSCs

2017 Fiscal Year Final Research Report

• Project/Area Number: 15K15624
• Research Category: Grant-in-Aid for Challenging Exploratory Research
• Allocation Type: Multi-year Fund
• Research Field: Otorhinolaryngology
• Research Institution: Keio University
• Principal Investigator: Fujioka Masato 慶應義塾大学, 医学部(信濃町), 講師 (70398626)
• Co-Investigator(Kenkyū-buntansha): 小川 郁 慶應義塾大学, 医学部, 教授 (00169179) ; 大石 直樹 慶應義塾大学, 医学部, 講師 (10348740)
• Project Period (FY): 2015-04-01 – 2018-03-31
• Keywords: 疾患iPS創薬 / WFS1 / Wolfram症候群 / 遺伝性難聴 / コモンマーモセット / 橋渡し研究 / ヒトiPS細胞

Outline of Final Research Achievements

We identified the expression pattern of WFS1 gene in the primate cochlea using a small non-human primate common marmoset (Suzuki N, 2016). In marmosets, unlike rodents, WFS1 was also expressed in the basal cell of stria vascularis, where severe atrophy was reported in the temporal bone of Wolfram syndrome patients. Based on these findings, we generated WFS1 positive cochlear cells from human iPSCs and its function was analyzed.

Free Research Field

耳科学