Evaluation of autoinflammatory responses in mouse cochlea

• Project/Area Number: 15H06255
• Research Category: Grant-in-Aid for Research Activity Start-up
• Allocation Type: Single-year Grants
• Research Field: Otorhinolaryngology
• Research Institution: Hamamatsu University School of Medicine
• Principal Investigator: Nakanishi Hiroshi 浜松医科大学, 医学部, 助教 (20444359)
• Project Period (FY): 2015-08-28 – 2017-03-31
• Project Status: Completed (Fiscal Year 2016)
• Budget Amount: ¥2,470,000 (Direct Cost: ¥1,900,000、Indirect Cost: ¥570,000); Fiscal Year 2016: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000); Fiscal Year 2015: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
• Keywords: 蝸牛 / 免疫応答

Outline of Final Research Achievements

NLRP3 protein is expressed in innate immune cells including macrophages. Gain of function mutations of NLRP3 induce the activation of NLRP3 inflammasome, resulting in the secretion of proinflammatory cytokine, interleukin-1 beta, to cause systemic inflammatory diseases, cryopyrin associated periodic syndromes. We found that a gain of function mutation of NLRP3 also caused non-syndromic hearing loss. We hypothesized that the hearing loss was caused by the activation of NLRP3 inflammasome mainly in the cochleae. We revealed that tissue-resident macrophage-like cells existed and mRNAs consisting NLRP3 inflammasome were expressed in the wild type mouse cochleae.

Research Products

• [Journal Article] 結核性中耳炎の1例 (2017)
  • Author(s): 中西啓, 泉智沙子，遠藤志織，大和谷崇，峯田周幸
  • Journal Title: JOHNS Volume: 32 Pages: 123-126
• [Journal Article] 残存上皮が外表化して治癒した先天性鼓膜真珠腫例 (2016)
  • Author(s): 中西啓, 水田邦博, 遠藤志織，細川久美子，大和谷崇，峯田周幸
  • Journal Title: 耳鼻咽喉科臨床 Volume: 32 Pages: 403-406
  • Peer Reviewed
• [Journal Article] 鼓膜炎 (2016)
  • Author(s): 中西啓, 水田邦博
  • Journal Title: JOHNS Volume: 32 Pages: 123-126
• [Journal Article] 高齢者における耳管開放症例 (2016)
  • Author(s): 中西啓, 水田邦博, 峯田周幸
  • Journal Title: JOHNS Volume: 32 Pages: 403-406
• [Journal Article] 診療所における高齢者の栄養補給・補液 (2016)
  • Author(s): 中西啓, 兼子恵美, 水田邦博
  • Journal Title: ENTONI Volume: 190 Pages: 59-67
• [Journal Article] TMC1 and TMC2 Localize at the Site of Mechanotransduction in Mammalian Inner Ear Hair Cell Stereocilia (2015)
  • Author(s): Kurima K, Ebrahim S, Pan B, Sedlacek M, Sengupta P, Millis B, Cui R, Nakanishi H, Fujikawa T, Kawashima Y, Choi BY, Monahan K, Holt JR, Griffith AJ, Kachar B
  • Journal Title: Cell Report Volume: 12 Issue: 10 Pages: 1606
  • DOI: 10.1016/j.celrep.2015.07.058
  • Peer Reviewed / Open Access / Int'l Joint Research
• [Presentation] NLRP3 mutation and cochlear autoinflammation cause syndromic and nonsyndromic hearing loss DFNA34 (2017)
  • Author(s): Nakanishi H, Kawashima Y, Kurima K, Chae JJ, Ross AM, Pinto-Patarroyo G, Patel SK, Muskett JA, Ratay JS, Chattaraj P, Grevich S, Brewer CC, Hoa M, Kim HJ, Butman JA, Broderick L, Hoffman HM, Aksentijevich I, Kastner DL, Goldbach-Mansky R, Griffith AJ
  • Organizer: ARO 40th Annual MidWinter Meeting
  • Place of Presentation: Baltimore Marriott Waterfront
  • Year and Date: 2017-02-11
  • Int'l Joint Research
• [Presentation] 本邦アッシャー症候群タイプ1症例の検討 (2016)
  • Author(s): 中西啓，遠藤志織，大和谷崇，水田邦博，峯田周幸
  • Organizer: 第61回日本聴覚医学会総会・学術講演会
  • Place of Presentation: 盛岡ニューシティーホテル
  • Year and Date: 2016-10-19
• [Presentation] COL4A5に遺伝子変異を同定することができたアルポート症候群例における聴力像の検討 (2016)
  • Author(s): 中西啓，遠藤志織，大和谷崇，水田邦博，峯田周幸
  • Organizer: 第26回日本耳科学会総会・学術講演会
  • Place of Presentation: アイランドホテル
  • Year and Date: 2016-10-05
• [Presentation] 当院におけるANCA関連血管炎性中耳炎症例の検討 (2016)
  • Author(s): 中西啓，遠藤志織，細川久美子，大和谷崇，水田邦博，峯田周幸
  • Organizer: 第64回耳鼻咽喉科学会中部地方部会連合会
  • Place of Presentation: 津都ホテル
  • Year and Date: 2016-07-15
• [Presentation] 先天性外耳道鼓膜真珠腫例 (2016)
  • Author(s): 中西啓，遠藤志織，大和谷崇，水田邦博，峯田周幸
  • Organizer: 第11回日本小児耳鼻咽喉科学会総会・学術講演会
  • Place of Presentation: ホテルクレメント徳島
  • Year and Date: 2016-06-30
• [Presentation] 高齢者の耳管開放症例 (2016)
  • Author(s): 中西啓，水田邦博，遠藤志織，大和谷崇，峯田周幸
  • Organizer: 第78回耳鼻咽喉科臨床学会総会・学術講演会
  • Place of Presentation: 城山観光ホテル
  • Year and Date: 2016-06-23
• [Presentation] 本邦アッシャー症候群タイプ2患者における聴力像の検討 (2015)
  • Author(s): 中西 啓，遠藤志織，水田邦博，峯田周幸
  • Organizer: 第60回日本聴覚医学会総会・学術講演会
  • Place of Presentation: 京王プラザホテル
  • Year and Date: 2015-10-21
• [Presentation] 蝸牛有毛細胞におていTmc2はTmc1を補完するのか (2015)
  • Author(s): 中西 啓，来間清人，峯田周幸
  • Organizer: 第25回日本耳科学会総会・学術講演会
  • Place of Presentation: 長崎ブリックホール
  • Year and Date: 2015-10-07