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北尻 真一郎  キタジリ シンイチロウ

教員組織電話番号
教育組織医学部 医学科 人工聴覚器学(寄附講座)FAX番号
職名特任准教授(寄附講座)メールアドレス
住所〒390-8621 長野県松本市旭3-1-1ホームページURL

プロフィール

研究分野
細胞生物学
人類遺伝学
耳鼻咽喉科学
キーワード:耳科学 , 細胞生物学 , 遺伝学
現在の研究課題
遺伝性難聴からの聴覚受容分子機構の解明

内耳におけるアクチンの動態解明

内耳機能とタイトジャンクション

所属学会
所属学会
日本人類遺伝学会
日本耳科学会
日本細胞生物学会
日本耳鼻咽喉科学会
耳鼻咽喉科臨床学会
日本気管食道科学会
学歴
取得学位
医学博士 , 京都大学
受賞学術賞
2009 , 日本細胞生物学会 若手優秀発表賞
2003 , Spoendlin's Junior Award of 40th Inner Ear Biology Meeting
研究職歴等
研究職歴
2009- , 京都大学 医学部附属病院 耳鼻咽喉科 助教
2008- , 京都大学 医学部附属病院 医員
2006- , 日本学術振興会 海外特別研究員
2005- , 米国立衛生研究所/国立聴覚・伝達障害研究所、分子遺伝学教室 博士研究員

研究活動業績

研究業績(著書・
発表論文等)
論文
TRIOBP-5 sculpts stereocilia rootlets and stiffens supporting cells enabling hearing
J Clin Invest Insight,4(12) 2019(Jun. 20)
Author:Tatsuya Katsuno, Inna A. Belyantseva, Alexander X. Cartagena-Rivera, Keisuke Ohta, Shawn M. Crump, Ronald S. Petralia, Kazuya Ono, Risa Tona, Ayesha Imtiaz, Atteeq Rehman, Hiroshi Kiyonari, Mari Kaneko, Ya-Xian Wang, Takaya Abe, Makoto Ikeya, Cristina Fenollar-Ferrer, Gavin P. Riordan, Elisabeth Wilson, Tracy S. Fitzgerald, Kohei Segawa, Koichi Omori, Juichi Ito, Gregory I. Frolenkov, Thomas B. Friedman, Shin-ichiro Kitajiri


難聴の遺伝カウンセリング
耳鼻咽喉科臨床,111(3):212-213 2018(Mar.)
Author:北尻真一郎 宇佐美真一


A Novel Splice Site Mutation of Myosin VI in Mice Leads to Stereociliary Fusion Caused by Disruption of Actin Networks in the Apical Region of Inner Ear Hair Cells
PLoS One,12(8):e0183477 2017(Aug. 23)
Author:Yuta Seki1, Yuki Miyasaka, Sari Suzuki, Kenta Wada, Shumpei P. Yasuda, Kunie Matsuoka, Yasuhiro Ohshiba, Kentaro Endo, Rie Ishii, Hiroshi Shitara, Shin-ichiro Kitajiri, Naomi Nakagata, Hirohide Takebayashi, Yoshiaki Kikkawa


Microarray analyses of otospheres derived from the cochlea in the inner ear identify putative transcription factors that regulate the characteristics of otospheres.
PLoS One,12(6):e0179901 2017(Jun.)
Author:Iki T, Tanaka M, Kitajiri SI, Kita T, Kawasaki Y, Mizukoshi A, Fujibuchi W, Nakagawa T, Nakahata T, Ito J, Omori K, Saito MK.


Constitutive activation of DIA1 (DIAPH1) via C-terminal truncation causes human sensorineural hearing loss.
EMBO Mol Med,8(11):1310-1324 2016(Nov.)
Author:Ueyama T, Ninoyu Y, Nishio SY, Miyoshi T, Torii H, Nishimura K, Sugahara K, Sakata H, Thumkeo D, Sakaguchi H, Watanabe N, Usami SI, Saito N, Kitajiri SI.


段階的分化誘導法を用いたヒトiPS細胞からの内耳有毛細胞様細胞の誘導
耳鼻咽喉科ニューロサイエンス,30:40-43 2016(May)
Author:大西 弘恵, Skerleva Desislava, 北尻 真一郎, 坂本 達則, 山本 典生, 伊藤 壽一, 中川 隆之


Tricellular Tight Junctions in the Inner Ear
BioMed research international,2016:6137541 2016
Author:Kitajiri S, Katsuno T


Carrier frequency of the GJB2 mutations that cause hereditary hearing loss in the Japanese population
Journal of human genetics,60(10):613-617 2015(Oct.)
Author:Taniguchi M, Matsuo H, Shimizu S, Nakayama A, Suzuki K, Hamajima N, Shinomiya N, Nishio S, Kosugi S, Usami S, Ito J, Kitajiri S


Limited hair cell induction from human induced pluripotent stem cells using a simple stepwise method
Neuroscience letters,599:49-54 2015(Jul.)
Author:Ohnishi H, Skerleva D, Kitajiri S, Sakamoto T, Yamamoto N, Ito J, Nakagawa T


Detailed hearing and vestibular profiles in the patients with COCH mutations
The Annals of otology, rhinology, and laryngology,124 Suppl 1:100S-10S 2015(May)
Author:Tsukada K, Ichinose A, Miyagawa M, Mori K, Hattori M, Nishio SY, Naito Y, Kitajiri S, Usami S


Mutational spectrum and clinical features of patients with ACTG1 mutations identified by massively parallel DNA sequencing
The Annals of otology, rhinology, and laryngology,124 Suppl 1:84S-93S 2015(May)
Author:Miyagawa M, Nishio SY, Ichinose A, Iwasaki S, Murata T, Kitajiri S, Usami S


The actin-bundling protein TRIOBP-4 and -5 promotes the motility of pancreatic cancer cells
Cancer letters,356(2 Pt B):367-373 2015(Jan.)
Author:Bao J, Wang S, Gunther LK, Kitajiri S, Li C, Sakamoto T


Downsloping high-frequency hearing loss due to inner ear tricellular tight junction disruption by a novel ILDR1 mutation in the Ig-like domain
PloS one,10(2):e0116931 2015
Author:Kim NK, Higashi T, Lee KY, Kim AR, Kitajiri S, Kim MY, Chang MY, Kim V, Oh SH, Kim D, Furuse M, Park WY, Choi BY


Deficiency of angulin-2/ILDR1, a tricellular tight junction-associated membrane protein, causes deafness with cochlear hair cell degeneration in mice
PloS one,10(3):e0120674 2015
Author:Higashi T, Katsuno T, Kitajiri S, Furuse M


Deafness in occludin-deficient mice with dislocation of tricellulin and progressive apoptosis of the hair cells
Biology open,3(8):759-766 2014(Jul.)
Author:Kitajiri S, Katsuno T, Sasaki H, Ito J, Furuse M, Tsukita S


Caprice/MISP is a novel F-actin bundling protein critical for actin-based cytoskeletal reorganizations
Genes to cells : devoted to molecular & cellular mechanisms,19(4):338-349 2014(Apr.)
Author:Kumeta M, Gilmore JL, Umeshima H, Ishikawa M, Kitajiri S, Horigome T, Kengaku M, Takeyasu K


In vivo imaging of mouse cochlea by optical coherence tomography
Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology,35(2):e84-9 2014(Feb.)
Author:Tona Y, Sakamoto T, Nakagawa T, Adachi T, Taniguchi M, Torii H, Hamaguchi K, Kitajiri S, Ito J


Ethnic differences in ATP-binding cassette transporter, sub-family G, member 2 (ABCG2/BCRP): genotype combinations and estimated functions
Drug metabolism and pharmacokinetics,29(6):490-492 2014
Author:Sakiyama M, Matsuo H, Takada Y, Nakamura T, Nakayama A, Takada T, Kitajiri S, Wakai K, Suzuki H, Shinomiya N


R1 motif is the major actin-binding domain of TRIOBP-4
Biochemistry,52(31):5256-5264 2013(Aug.)
Author:Bao J, Bielski E, Bachhawat A, Taha D, Gunther LK, Thirumurugan K, Kitajiri S, Sakamoto T


Analysis of the 'angulin' proteins LSR, ILDR1 and ILDR2--tricellulin recruitment, epithelial barrier function and implication in deafness pathogenesis
Journal of cell science,126(Pt 4):966-977 2013(Feb.)
Author:Higashi T, Tokuda S, Kitajiri S, Masuda S, Nakamura H, Oda Y, Furuse M


Adipose tissue-derived stromal cells protect hair cells from aminoglycoside
The Laryngoscope,121(6):1281-1286 2011(Jun.)
Author:Yoshida A, Kitajiri S, Nakagawa T, Hashido K, Inaoka T, Ito J


Insulin-like growth factor 1 protects vestibular hair cells from aminoglycosides
Neuroreport,22(1):38-43 2011(Jan.)
Author:Angunsri N, Taura A, Nakagawa T, Hayashi Y, Kitajiri S, Omi E, Ishikawa K, Ito J


Actin-bundling protein TRIOBP forms resilient rootlets of hair cell stereocilia essential for hearing
Cell,141(5):786-798 2010(May)
Author:Kitajiri S, Sakamoto T, Belyantseva IA, Goodyear RJ, Stepanyan R, Fujiwara I, Bird JE, Riazuddin S, Riazuddin S, Ahmed ZM, Hinshaw JE, Sellers J, Bartles JR, Hammer JA 3rd, Richardson GP, Griffith AJ, Frolenkov GI, Friedman TB


Identities, frequencies, and origins of TMC1 mutations causing DFNB7/B11 deafness in Pakistan
Clin Genet,72(6):546-550 2007
Author:Kitajiri SI, McNamara R, Makishima T, Husnain T, Zafar AU, Kittles RA, Ahmed ZM, Friedman TB, Riazuddin S, Griffith AJ


A novel mutation at the DFNA36 hearing loss locus reveals a critical function and potential genotype-phenotype correlation for amino acid-572 of TMC1
Clin Genet,71(2):148-152 2007
Author:Kitajiri S, Makishima T, Friedman TB, Griffith AJ


Mutations of the RDX gene cause nonsyndromic hearing loss at the DFNB24 locus
Hum Mutat,28(5):417-423 2007
Author:Khan SY, Ahmed ZM, Shabbir MI, Kitajiri S, Kalsoom S, Tasneem S, Shayiq S, Ramesh A, Srisailpathy S, Khan SN, Smith RJ, Riazuddin S, Friedman TB, Riazuddin S


Tricellulin is a tight-junction protein necessary for hearing
Am J Hum Genet,79(6):1040-1051 2006
Author:Riazuddin S, Ahmed ZM, Fanning AS, Lagziel A, Kitajiri S, Ramzan K, Khan SN, Chattaraj P, Friedman PL, Anderson JM, Belyantseva IA, Forge A, Riazuddin S, Friedman TB


Disruption and restoration of cell?cell junctions in mouse vestibular epithelia following aminoglycoside treatment
Hear Res,205( 1-2):201-209 2005
Author:Kim TS, Nakagawa T, Kitajiri S, Endo T, Takebayashi S, Iguchi F, Kita T, Tamura T, Ito J


Genes related to hearing disorders
Acta Otolaryngologica,Suppl. 551:10-13 2004
Author:Kitajiri S, Sakamoto T, Ito J


Expression patterns of claudins, tight junction adhesion molecules, in the inner ear
Hear Res,187( 1-2):25-34 2004
Author:Kitajiri SI, Furuse M, Morita K, Saishin-Kiuchi Y, Kido H, Ito J, Tsukita S


Radixin deficiency causes deafness associated with progressive degeneration of cochlear stereocilia
Journal of Cell Biology,166(4):559-570 2004
Author:Kitajiri S, Fukumoto K, Hata M, Sasaki H, Katsuno T, Nakagawa T, Ito J, Tsukita S, Tsukita S


Compartmentalization established by claudin-11-based tight junctions in stria vascularis is required for hearing through generation of endocochlear potential
Journal of Cell Science,117(Pt 21):5087-5096 2004
Author:Kitajiri S, Miyamoto T, Mineharu A, Sonoda N, Furuse K, Hata M, Sasaki H, Mori Y, Kubota T, Ito J, Furuse M, Tsukita S


The presence of large lymph node metastasis as a prognostic factor of papillary thyroid carcinoma
Auris Nasus Larynx,30(2):169-174 2003
Author:Kitajiri S, Hiraumi H, Hirose T, Hosaka N


Increased expression of integrin beta-4 in papillary thyroid carcinoma with gross lymph node metastasis
Pathology international,52(7):438-441 2002(Jul.)
Author:Kitajiri S, Hosaka N, Hiraumi H, Hirose T, Ikehara S


Is Corticosteroid Therapy Effective for Sudden-Onset Sensorineural Hearing Loss at Lower Frequencies?
Arch Otolaryngol Head Neck Surg,128(4):365-367 2002
Author:Kitajiri S, Tabuchi K, Hiraumi H, Hirose T


Ectopic pituitary adenoma with malignant transformation
American Journal of Surgical Pathology,26(8):1078-1082 2002
Author:Hosaka N, Kitajiri S, Hiraumi H, Nogaki H, Toki J, Yang G, Hisha H, Ikehara S


Radiosensitive hemangiopericytoma in the soft palate
Auris Nasus Larynx,9(1):95-97 2002
Author:Hiraumi H, Kitajiri S, Hirose T, Hosaka N, Toki J, Ikehara S


Dermal thymus: case report and review of the literature
American journal of otolaryngology,22(4):294-296 2001(Jul.)
Author:Hiraumi H, Tabuchi K, Kitajiri S


Relief of Post-tonsillectomy Pain by Release of Lidocaine from Fibrin Glue
Laryngoscope,111(5):642-644 2001
Author:Kitajiri S, Tabuchi K, Hiraumi H, Kaetsu H


Transnasal Bamboo Foreign Body Lodged in the Sphenoid Sinus
Auris Nasus Larynx,28(4):365-367 2001
Author:Kitajiri S, Tabuchi K, Hiraumi H


A Case Report of FSH-Producing Nasal Ectopic Pituitary Adenoma Extending to the Frontal Cranial Fossa
Arch Otolaryngol Head Neck Surg,126(6):782-784 2000
Author:Kitajiri S, Hosaka N, Tabuchi K, Hiraumi H, Nogaki H, Tatsumi S, Toki J, Ikehara S


講演・口頭発表等
ヒトiPS細胞を用いたミトコンドリア1555変異難聴の病態解明
第28回日本耳科学会 2018(Oct.)
Presenter:喜多知子、伊木健浩、水越彬文、宇佐美真一、北尻真一郎


アブミ骨奇形の1家系の臨床調査
第28回日本耳科学会 2018(Oct.)
Presenter:堀龍介、児嶋剛、岡上雄介、藤村真太郎、鹿子島大貴、田口敦士、北尻真一郎、庄司和彦


OTOF遺伝子p.R1172Q変異による難聴発症機序の解析
第63回日本聴覚医学会 2018(Oct.)
Presenter:岩佐陽一郎、北尻真一郎、西尾信哉、吉村豪兼、宇佐美真一


ACTG1遺伝子変異による難聴症例の検討
第63回日本聴覚医学会 2018(Oct.)
Presenter:宮嶋宏樹、茂木英明、北尻真一郎、西尾信哉、村田考啓、池園哲郎、武田英彦、阿部聡子、岩崎聡、高橋優宏、内藤泰、山崎博司、神田幸彦、宇佐美真一


Regulation of the localization of molecules in hair cell stereocilia by TRIOBP
ARO The 41st Annual MidWinter Meeting 2018(Feb. 09)
Presenter:Tomoko Kita, Raj K Ladher, Shin-ichi Usami, Shin-ichiro Kitajiri


Making tool for analysis of TRIOBP function using induced pluripotent stem cell
ARO The 41st Annual MidWinter Meeting 2018(Feb. 09)
Presenter:Hiroe Ohnishi、Shin-ichiro Kitajiri、Xiangxin Lou、Akiko Taura、Mirei Taniguchi、Fumi Ebisu、Tatsunori Sakamoto、Norio Yamamoto、Juichi Ito1、Koichi Omori、Takayuki Nakagawa


不動毛の「根」の低形成と難聴
第27回日本耳科学会 2017(Nov. 22)
Presenter:北尻真一郎、伊藤壽一


遺伝性難聴DFNA1の原因遺伝子DIAPH1と、その変異による難聴発症メカニズムの解明
日本人類遺伝学会第62回大会 2017(Nov. 15)
Presenter:北尻真一郎、二之湯弦、西尾信哉、三好拓志、鳥居紘子、西村幸司、菅原一真、坂田英明、Dean Thumkeo、坂口博史、渡邊直樹、宇佐美真一、齋藤尚亮、上山健彦


An Attempt to Establish In Vitro Model Using TRIOBP KO Induced Pluripotent Stem Cells
International Symposium on Inner Ear Therapies 2017(Nov. 01)
Presenter:Hiroe Ohnishi、Shin-ichiro Kitajiri、Xiangxin Lou、Akiko Taura、Mirei Taniguchi、Fumi Ebisu、Tatsunori Sakamoto、Norio Yamamoto、Juichi Ito1、Koichi Omori、Takayuki Nakagawa


The development and maintenance of hair cell stereocilia rootlets by isoform specific functions of TRIOBP
54th workshop on Inner Ear Biology and Symposium 2017(Sep. 13)
Presenter:Tatsuya Katsuno, Inna A. Belyantseva, Ronald S. Petralia, Ya-Xian Wang, Keisuke Ohta, Kazuya Ono, Makoto Ikeya, Gavin P. Riordan, Joseph Duda, Elizabeth Wilson, Tracy Fitzgerald, Atteeq U. Rehman, Ayesha Imtiaz, Juichi Ito, Thomas B. Friedman, Shin-ichiro Kitajiri


Abnormal actin elongation activity of a novel hearing-loss Dia1 mutant revealed by single-molecule speckle microscopy
54th workshop on Inner Ear Biology and Symposium 2017(Sep. 13)
Presenter:Takushi Miyoshi, Yuzuru Ninoyu, Naoki Watanabe, Shin-ichiro Kitajiri, and Takehiko Ueyama


The functional analysis of DIAPH1, the responsible gene for DFNA1
International Congress of ORL-HNS 2017(Apr. 21)
Presenter:Shin-ichiro KITAJIRI


DIA1変異マウスを用いたDFNA1の病態解明
日本耳鼻咽喉科学会会報 120 4 521 2017(Apr.)
Presenter:二之湯 弦, 北尻 真一郎, 坂口 博史, 宇佐美 真一, 齋藤 尚亮, 上山 健彦


変異DIA1によるヒト遺伝性難聴DFNA1の病態解明
Otology Japan 26 4 236 2016(Sep.)
Presenter:北尻 真一郎


常染色体劣性遺伝形式をとるGJB2遺伝子変異の日本人における保因者頻度
Otology Japan 26 4 622 2016(Sep.)
Presenter:北尻 真一郎, 谷口 美玲, 西尾 信哉, 宇佐美 真一, 大森 孝一

研究費
科学研究費補助金(研究代表者)
2017 - 2019 , ヒト遺伝性難聴を切り口とする、聴覚生理の網羅的解析 , 基盤研究(B)