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J-GLOBAL ID：201702257733900857   Reference number：17A1099635

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

疾患特異的iPSCを明らかにする変性表現型を用いた蝸牛細胞モデリングと先天性進行性難聴の治療示唆【Powered by NICT】

Author (14)： Hosoya Makoto (Department of Otorhinolaryngology, Head and Neck Surgery, Keio University School of Medicine, 35 Shinanomachi Shinjyuku-ku, Tokyo 160-8582, Japan) ,  Fujioka Masato (Department of Otorhinolaryngology, Head and Neck Surgery, Keio University School of Medicine, 35 Shinanomachi Shinjyuku-ku, Tokyo 160-8582, Japan) ,  Sone Takefumi (Department of Physiology, Keio University School of Medicine, 35 Shinanomachi Shinjyuku-ku, Tokyo 160-8582, Japan) ,  Okamoto Satoshi (Department of Physiology, Keio University School of Medicine, 35 Shinanomachi Shinjyuku-ku, Tokyo 160-8582, Japan) ,  Akamatsu Wado (Department of Physiology, Keio University School of Medicine, 35 Shinanomachi Shinjyuku-ku, Tokyo 160-8582, Japan) ,  Akamatsu Wado (Center for Genomic and Regenerative Medicine, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan) ,  Ukai Hideki (Laboratory for Synthetic Biology, RIKEN Quantitative Biology Center, 2-2-3 Minatojima-minamimachi, Chuo-ku, Kobe, Hyogo 650-0047, Japan) ,  Ueda Hiroki R. (Laboratory for Synthetic Biology, RIKEN Quantitative Biology Center, 2-2-3 Minatojima-minamimachi, Chuo-ku, Kobe, Hyogo 650-0047, Japan) ,  Ueda Hiroki R. (Department of Systems Pharmacology, Graduate School of Medicine, The University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-0033, Japan) ,  Ueda Hiroki R. (CREST, Japan Science and Technology Agency, 4-1-8 Honcho, Kawaguchi, Saitama 332-0012, Japan) ,  Ogawa Kaoru (Department of Otorhinolaryngology, Head and Neck Surgery, Keio University School of Medicine, 35 Shinanomachi Shinjyuku-ku, Tokyo 160-8582, Japan) ,  Matsunaga Tatsuo (The Laboratory of Auditory Disorders and Division of Hearing and Balance Research, National Institute of Sensory Organs, National Tokyo Medical Center, 2-5-1 Higashigaoka, Meguro-ku, Tokyo 152-8902, Japan) ,  Matsunaga Tatsuo (Medical Genetics Center, National Tokyo Medical Center, 2-5-1 Higashigaoka, Meguro-ku, Tokyo 152-8902, Japan) ,  Okano Hideyuki (Department of Physiology, Keio University School of Medicine, 35 Shinanomachi Shinjyuku-ku, Tokyo 160-8582, Japan)
Material： Cell Reports
Volume： 18  Issue： 1  Page： 68-81  Publication year： 2017 
JST Material Number： W3124A  ISSN： 2211-1247  Document type： Article
Article type： 原著論文  Country of issue： Netherlands (NLD)  Language： ENGLISH (EN)

Abstract/Point： Hearing impairments are the mo...

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Thesaurus term： pathogenesis ,  gene ,  Rodentia ,  Cell Death ,  *Phenotype ,  *therapy ,  *cochlea ,  induced pluripotent stem cell ,  hearing disorder ,  experimental disease ,  epithelial cell ,  pendrin ,  anion exchanger ,  Mammalia
Semi thesaurus term： Pendred症候群 ,  遺伝性難聴 , 【Automatic Indexing@JST】

Author keywords (4)： Pendred syndrome ,  disease-specific iPS cells ,  inner ear ,  outer sulcus cells

JST classification (3)： Congenital diseases,deformities in general.  (GD03010V) ,  Diseases of ear,nose,pharynx and larynx  (GQ03000X) ,  Basic otorhinolaryngology  (GQ01020F)

Terms in the title (9)： 疾患 ,  IPSC ,  変性 ,  表現型 ,  蝸牛 ,  細胞 ,  モデリング ,  進行性難聴 ,  治療