Cochlear Cell Modeling Using Disease-Specific iPSCs Unveils a Degenerative Phenotype and Suggests Treatments for Congenital Progressive Hearing Loss

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• 作者：Makoto Hosoya¹ ; Masato Fujioka¹ ; Takefumi Sone² ; Satoshi Okamoto² ; Wado Akamatsu² ; ³ ; Hideki Ukai⁴ ; Hiroki R. Ueda⁴ ; ⁵ ; ⁶ ; Kaoru Ogawa¹ ; Tatsuo Matsunaga⁷ ; ⁸ ; Hideyuki Okano² ; ⁹ ; ^{hidokano@a2.keio.jp}
• 关键词：Pendred syndrome ; disease-specific iPS cells ; inner ear ; outer sulcus cells
• 刊名：Cell Reports
• 出版年：2017
• 出版时间：3 January 2017
• 年：2017
• 卷：18
• 期：1
• 页码：68-81
• 全文大小：5940 K
• 卷排序：18

文摘

In vitro human cochlear cell model is established and used for drug development Pendred syndrome (PDS)-specific iPSCs explain the progressive symptoms of PDS hiPSCs present intracellular aggregations and cellular vulnerability PDS iPSCs reveal a potential treatment strategy for progressive hearing loss