北尻 真一郎 (キタジリ シンイチロウ)
医学部 医学科 人工聴覚器学（寄附講座）
特任准教授（寄附講座）
研究者情報
研究活動情報
論文
- Human deafness-associated variants alter the dynamics of key molecules in hair cell stereocilia F-actin cores.
Miyoshi T, Belyantseva IA, Kitajiri SI, Miyajima H, Nishio SY, Usami SI, Kim BJ, Choi BY, Omori K, Shroff H, Friedman TB.
Hum Genet., 141(3_4), 363-382, 2022年
- Prevalence and Clinical Characteristics of Hearing Loss Caused by MYH14 Variants.
Hiramatsu K, Nishio SY, Kitajiri SI, Kitano T, Moteki H, Usami SI, On Behalf Of The Deafness Gene Study Consortium.
Genes(Basel)., 12(10), 1623, 2021年 - 不動毛の「根」を形成する分子TRIOBPの発見
北尻真一郎
Otol Jpn, 31(3), 221-224, 2021年 - The use of a MITO-Porter to deliver exogenous therapeutic RNA to a mitochondrial disease's cell with a A1555G mutation in the mitochondrial 12S rRNA gene results in an increase in mitochondrial respiratory activity
Yamada Y, Maruyama M, Kita T, Usami SI, Kitajiri SI, Harashima H.
Mitochondrion, 55, 134-144, 2020年11月 - Novel ACTG1 mutations in patients identified by massively parallel DNA sequencing cause progressive hearing loss
Miyajima H, Moteki H, Day T, Nishio SY, Murata T, Ikezono T, Takeda H, Abe S, Iwasaki S, Takahashi M, Naito Y, Yamazaki H, Kanda Y, Kitajiri SI, Usami SI.
Sci Rep, 10(1), 7056, 2020年04月 - Digenic inheritance of mutations in EPHA2 and SLC26A4 in Pendred syndrome
Li M, Nishio SY, Naruse C, Riddell M, Sapski S, Katsuno T, Hikita T, Mizapourshafiyi F, Smith FM, Cooper LT, Lee MG, Asano M, Boettger T, Krueger M, Wietelmann A, Graumann J, Day BW, Boyd AW, Offermanns S, Kitajiri SI, Usami SI, Nakayama M.
Nat Commun, 11(1), 1343, 2020年03月 - Clinical Characteristics and In Vitro Analysis of MYO6 Variants Causing Late-Onset Progressive Hearing Loss
Oka SI, Day TF, Nishio SY, Moteki H, Miyagawa M, Morita S, Izumi S, Ikezono T, Abe S, Nakayama J, Hyogo M, Okamoto N, Uehara N, Oshikawa C, Kitajiri SI, Usami SI.
Genes (Basel), 11(3), 273, 2020年03月 - Mid-Frequency Hearing Loss Is Characteristic Clinical Feature of OTOA-Associated Hearing Loss
Kenjiro Sugiyama, Hideaki Moteki, Shin-ichiro Kitajiri, Tomohiro Kitano, Shin-ya Nishio, Tomomi Yamaguchi, Keiko Wakui, Satoko Abe, Akiko Ozaki, Remi Motegi, Hirooki Matsui, Masato Teraoka, Yumiko Kobayashi, Tomoki Kosho, Shin-ichi Usami
Genes, 10(9), 2019年09月16日 - Detailed Clinical Features of Deafness Caused by a Claudin-14 Variant
Tomohiro Kitano, Shin-ichiro Kitajiri, Shin-ya Nishio and Shin-ichi Usami
Int. J. Mol. Sci., 20(18), 2019年09月16日 - Differential disruption of autoinhibition and defect in assembly of cytoskeleton during cell division decide the fate of human DIAPH1-related cytoskeletopathy
Bong Jik Kim, Takehiko Ueyama, takushi Miyoshi, Seungmin lee, Jin Hee Han, Hye-rim Park, Ah reum Kim, Jayoung Oh, Min Young Kim, Yong Seok Kang, Doo Yi Oh, Jiwon Yun, Sang Mee Hwang, Nayoung K D Kim, Woong-Yang Park, Shin-ichiro Kitajiri, Byung Yoon Choi
Journal of Medical Genetics, 2019年08月31日 - TRIOBP-5 sculpts stereocilia rootlets and stiffens supporting cells enabling hearing
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
J Clin Invest Insight, 4(12), 2019年06月20日 - 知っておきたい遺伝学的検査と遺伝外来のABC. 遺伝子変異による耳鼻咽喉科疾患. 感音難聴
北尻真一郎、西尾信哉、宮川麻衣子、宇佐美真一
耳鼻咽喉科・頭頸部外科, 90(8), 632-638, 2018年07月20日 - 難聴の遺伝カウンセリング
北尻真一郎 宇佐美真一
耳鼻咽喉科臨床, 111(3), 212-213, 2018年03月 - 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
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
PLOS ONE, 12(8), e0183477, 2017年08月23日, 査読有り
- Microarray analyses of otospheres derived from the cochlea in the inner ear identify putative transcription factors that regulate the characteristics of otospheres.
Iki T, Tanaka M, Kitajiri SI, Kita T, Kawasaki Y, Mizukoshi A, Fujibuchi W, Nakagawa T, Nakahata T, Ito J, Omori K, Saito MK.
PLOS ONE, 12(6), e0179901, 2017年06月, 査読有り - Constitutive activation of DIA1 (DIAPH1) via C-terminal truncation causes human sensorineural hearing loss.
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.
EMBO Mol Med, 8(11), 1310-1324, 2016年11月, 査読有り - 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
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
耳鼻咽喉科ニューロサイエンス, 30, 40-43, 2016年05月 - Tricellular Tight Junctions in the Inner Ear
Kitajiri S, Katsuno T
BioMed Research International, 2016, 6137541, 2016年, 査読有り - Carrier frequency of the GJB2 mutations that cause hereditary hearing loss in the Japanese population
Taniguchi M, Matsuo H, Shimizu S, Nakayama A, Suzuki K, Hamajima N, Shinomiya N, Nishio S, Kosugi S, Usami S, Ito J, Kitajiri S
JOURNAL OF HUMAN GENETICS, 60(10), 613-617, 2015年10月, 査読有り - Limited hair cell induction from human induced pluripotent stem cells using a simple stepwise method
Ohnishi H, Skerleva D, Kitajiri S, Sakamoto T, Yamamoto N, Ito J, Nakagawa T
NEUROSCIENCE LETTERS, 599, 49-54, 2015年07月, 査読有り - Mutational spectrum and clinical features of patients with ACTG1 mutations identified by massively parallel DNA sequencing
Miyagawa M, Nishio SY, Ichinose A, Iwasaki S, Murata T, Kitajiri S, Usami S
The Annals of otology, rhinology, and laryngology, 124 Suppl 1, 84S-93S, 2015年05月, 査読有り - Detailed hearing and vestibular profiles in the patients with COCH mutations
Tsukada K, Ichinose A, Miyagawa M, Mori K, Hattori M, Nishio SY, Naito Y, Kitajiri S, Usami S
The Annals of otology, rhinology, and laryngology, 124 Suppl 1, 100S-10S, 2015年05月, 査読有り - The actin-bundling protein TRIOBP-4 and -5 promotes the motility of pancreatic cancer cells
Bao J, Wang S, Gunther LK, Kitajiri S, Li C, Sakamoto T
CANCER LETTERS, 356(2 Pt B), 367-373, 2015年01月, 査読有り - Downsloping high-frequency hearing loss due to inner ear tricellular tight junction disruption by a novel ILDR1 mutation in the Ig-like domain
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
PLOS ONE, 10(2), e0116931, 2015年, 査読有り - Deficiency of angulin-2/ILDR1, a tricellular tight junction-associated membrane protein, causes deafness with cochlear hair cell degeneration in mice
Higashi T, Katsuno T, Kitajiri S, Furuse M
PLOS ONE, 10(3), e0120674, 2015年, 査読有り - Deafness in occludin-deficient mice with dislocation of tricellulin and progressive apoptosis of the hair cells
Kitajiri S, Katsuno T, Sasaki H, Ito J, Furuse M, Tsukita S
Biology open, 3(8), 759-766, 2014年07月, 査読有り - Caprice/MISP is a novel F-actin bundling protein critical for actin-based cytoskeletal reorganizations
Kumeta M, Gilmore JL, Umeshima H, Ishikawa M, Kitajiri S, Horigome T, Kengaku M, Takeyasu K
Genes to cells : devoted to molecular & cellular mechanisms, 19(4), 338-349, 2014年04月, 査読有り - In vivo imaging of mouse cochlea by optical coherence tomography
Tona Y, Sakamoto T, Nakagawa T, Adachi T, Taniguchi M, Torii H, Hamaguchi K, Kitajiri S, Ito J
Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology, 35(2), e84-9, 2014年02月, 査読有り - Ethnic differences in ATP-binding cassette transporter, sub-family G, member 2 (ABCG2/BCRP): genotype combinations and estimated functions
Sakiyama M, Matsuo H, Takada Y, Nakamura T, Nakayama A, Takada T, Kitajiri S, Wakai K, Suzuki H, Shinomiya N
DRUG METABOLISM AND PHARMACOKINETICS, 29(6), 490-492, 2014年, 査読有り - R1 motif is the major actin-binding domain of TRIOBP-4
Bao J, Bielski E, Bachhawat A, Taha D, Gunther LK, Thirumurugan K, Kitajiri S, Sakamoto T
Biochemistry, 52(31), 5256-5264, 2013年08月, 査読有り - Analysis of the 'angulin' proteins LSR, ILDR1 and ILDR2--tricellulin recruitment, epithelial barrier function and implication in deafness pathogenesis
Higashi T, Tokuda S, Kitajiri S, Masuda S, Nakamura H, Oda Y, Furuse M
JOURNAL OF CELL SCIENCE, 126(Pt 4), 966-977, 2013年02月, 査読有り - Adipose tissue-derived stromal cells protect hair cells from aminoglycoside
Yoshida A, Kitajiri S, Nakagawa T, Hashido K, Inaoka T, Ito J
The Laryngoscope, 121(6), 1281-1286, 2011年06月, 査読有り - Insulin-like growth factor 1 protects vestibular hair cells from aminoglycosides
Angunsri N, Taura A, Nakagawa T, Hayashi Y, Kitajiri S, Omi E, Ishikawa K, Ito J
NEUROREPORT, 22(1), 38-43, 2011年01月, 査読有り - Actin-bundling protein TRIOBP forms resilient rootlets of hair cell stereocilia essential for hearing
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
Cell, 141(5), 786-798, 2010年05月, 査読有り - Mutations of the RDX gene cause nonsyndromic hearing loss at the DFNB24 locus
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
Hum Mutat, 28(5), 417-423, 2007年, 査読有り - A novel mutation at the DFNA36 hearing loss locus reveals a critical function and potential genotype-phenotype correlation for amino acid-572 of TMC1
Kitajiri S, Makishima T, Friedman TB, Griffith AJ
Clin Genet, 71(2), 148-152, 2007年, 査読有り - Identities, frequencies, and origins of TMC1 mutations causing DFNB7/B11 deafness in Pakistan
Kitajiri SI, McNamara R, Makishima T, Husnain T, Zafar AU, Kittles RA, Ahmed ZM, Friedman TB, Riazuddin S, Griffith AJ
Clin Genet, 72(6), 546-550, 2007年, 査読有り - Tricellulin is a tight-junction protein necessary for hearing
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
Am J Hum Genet, 79(6), 1040-1051, 2006年, 査読有り - Disruption and restoration of cell?cell junctions in mouse vestibular epithelia following aminoglycoside treatment
Kim TS, Nakagawa T, Kitajiri S, Endo T, Takebayashi S, Iguchi F, Kita T, Tamura T, Ito J
Hear Res, 205( 1-2), 201-209, 2005年, 査読有り - Compartmentalization established by claudin-11-based tight junctions in stria vascularis is required for hearing through generation of endocochlear potential
Kitajiri S, Miyamoto T, Mineharu A, Sonoda N, Furuse K, Hata M, Sasaki H, Mori Y, Kubota T, Ito J, Furuse M, Tsukita S
JOURNAL OF CELL SCIENCE, 117(Pt 21), 5087-5096, 2004年, 査読有り - Radixin deficiency causes deafness associated with progressive degeneration of cochlear stereocilia
Kitajiri S, Fukumoto K, Hata M, Sasaki H, Katsuno T, Nakagawa T, Ito J, Tsukita S, Tsukita S
Journal of Cell Biology, 166(4), 559-570, 2004年, 査読有り - Expression patterns of claudins, tight junction adhesion molecules, in the inner ear
Kitajiri SI, Furuse M, Morita K, Saishin-Kiuchi Y, Kido H, Ito J, Tsukita S
Hear Res, 187( 1-2), 25-34, 2004年, 査読有り - Genes related to hearing disorders
Kitajiri S, Sakamoto T, Ito J
Acta Otolaryngologica, Suppl. 551, 10-13, 2004年, 査読有り - The presence of large lymph node metastasis as a prognostic factor of papillary thyroid carcinoma
Kitajiri S, Hiraumi H, Hirose T, Hosaka N
AURIS NASUS LARYNX, 30(2), 169-174, 2003年, 査読有り - Increased expression of integrin beta-4 in papillary thyroid carcinoma with gross lymph node metastasis
Kitajiri S, Hosaka N, Hiraumi H, Hirose T, Ikehara S
PATHOLOGY INTERNATIONAL, 52(7), 438-441, 2002年07月, 査読有り - Is Corticosteroid Therapy Effective for Sudden-Onset Sensorineural Hearing Loss at Lower Frequencies?
Kitajiri S, Tabuchi K, Hiraumi H, Hirose T
Arch Otolaryngol Head Neck Surg, 128(4), 365-367, 2002年, 査読有り - Radiosensitive hemangiopericytoma in the soft palate
Hiraumi H, Kitajiri S, Hirose T, Hosaka N, Toki J, Ikehara S
AURIS NASUS LARYNX, 9(1), 95-97, 2002年, 査読有り - Ectopic pituitary adenoma with malignant transformation
Hosaka N, Kitajiri S, Hiraumi H, Nogaki H, Toki J, Yang G, Hisha H, Ikehara S
AMERICAN JOURNAL OF SURGICAL PATHOLOGY, 26(8), 1078-1082, 2002年, 査読有り - Dermal thymus: case report and review of the literature
Hiraumi H, Tabuchi K, Kitajiri S
American journal of otolaryngology, 22(4), 294-296, 2001年07月, 査読有り - Relief of Post-tonsillectomy Pain by Release of Lidocaine from Fibrin Glue
Kitajiri S, Tabuchi K, Hiraumi H, Kaetsu H
LARYNGOSCOPE, 111(5), 642-644, 2001年, 査読有り - Transnasal Bamboo Foreign Body Lodged in the Sphenoid Sinus
Kitajiri S, Tabuchi K, Hiraumi H
AURIS NASUS LARYNX, 28(4), 365-367, 2001年, 査読有り - A Case Report of FSH-Producing Nasal Ectopic Pituitary Adenoma Extending to the Frontal Cranial Fossa
Kitajiri S, Hosaka N, Tabuchi K, Hiraumi H, Nogaki H, Tatsumi S, Toki J, Ikehara S
Arch Otolaryngol Head Neck Surg, 126(6), 782-784, 2000年, 査読有り
講演・口頭発表等
- ACTG1遺伝子変異による難聴症例の検討
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
第63回日本聴覚医学会, 2018年10月 - OTOF遺伝子p.R1172Q変異による難聴発症機序の解析
岩佐陽一郎、北尻真一郎、西尾信哉、吉村豪兼、宇佐美真一
第63回日本聴覚医学会, 2018年10月 - アブミ骨奇形の１家系の臨床調査
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
第28回日本耳科学会, 2018年10月 - 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
喜多知子、伊木健浩、水越彬文、宇佐美真一、北尻真一郎
第28回日本耳科学会, 2018年10月 - Making tool for analysis of TRIOBP function using induced pluripotent stem cell
Hiroe Ohnishi、Shin-ichiro Kitajiri、Xiangxin Lou、Akiko Taura、Mirei Taniguchi、Fumi Ebisu、Tatsunori Sakamoto、Norio Yamamoto、Juichi Ito1、Koichi Omori、Takayuki Nakagawa
ARO The 41st Annual MidWinter Meeting, 2018年02月09日 - Regulation of the localization of molecules in hair cell stereocilia by TRIOBP
Tomoko Kita, Raj K Ladher, Shin-ichi Usami, Shin-ichiro Kitajiri
ARO The 41st Annual MidWinter Meeting, 2018年02月09日 - 不動毛の「根」の低形成と難聴
北尻真一郎、伊藤壽一
第27回日本耳科学会, 2017年11月22日 - 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
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
日本人類遺伝学会第62回大会, 2017年11月15日 - An Attempt to Establish In Vitro Model Using TRIOBP KO Induced Pluripotent Stem Cells
Hiroe Ohnishi、Shin-ichiro Kitajiri、Xiangxin Lou、Akiko Taura、Mirei Taniguchi、Fumi Ebisu、Tatsunori Sakamoto、Norio Yamamoto、Juichi Ito1、Koichi Omori、Takayuki Nakagawa
International Symposium on Inner Ear Therapies, 2017年11月01日 - Abnormal actin elongation activity of a novel hearing-loss Dia1 mutant revealed by single-molecule speckle microscopy
Takushi Miyoshi, Yuzuru Ninoyu, Naoki Watanabe, Shin-ichiro Kitajiri, and Takehiko Ueyama
54th workshop on Inner Ear Biology and Symposium, 2017年09月13日 - The development and maintenance of hair cell stereocilia rootlets by isoform specific functions of TRIOBP
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
54th workshop on Inner Ear Biology and Symposium, 2017年09月13日 - The functional analysis of DIAPH1, the responsible gene for DFNA1
Shin-ichiro KITAJIRI
International Congress of ORL-HNS, 2017年04月21日, 招待有り - DIA1変異マウスを用いたDFNA1の病態解明
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
日本耳鼻咽喉科学会会報 120 4 521, 2017年04月 - 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
北尻 真一郎, 谷口 美玲, 西尾 信哉, 宇佐美 真一, 大森 孝一
Otology Japan 26 4 622, 2016年09月 - 変異DIA1によるヒト遺伝性難聴DFNA1の病態解明
北尻 真一郎
Otology Japan 26 4 236, 2016年09月
