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神谷 和作(カミヤ カズサク)

研究テーマ 難聴に対する新規細胞治療法の開発
研究業績(論文) Modeling Gap junction beta 2 gene-related deafness with human iPSC
Ichiro Fukunaga, Yoko Oe, Keiko Danzaki, Sayaka Ohta, Cheng Chen, Kyoko Shirai, Atsushi Kawano, Katsuhisa Ikeda, Kazusaku Kamiya
Human Molecular Genetics, 2021 doi: 10.1093/hmg/ddab097. Ahead of print
(読売新聞、時事通信 他)

Activin/Nodal/TGF-β Pathway Inhibitor Accelerates BMP4-Induced Cochlear Gap Junction Formation During in vitro Differentiation of Embryonic Stem Cells.
Fukunaga I, Oe Y, Chen C, Danzaki K, Ohta S, Koike A, Ikeda K, Kamiya K.
Front Cell Dev Biol. 2021 ;9:602197

Generation of two iPSC lines from siblings of a homozygous patient with hearing
loss and a heterozygous carrier with normal hearing carrying p.G45E/Y136X mutation in GJB2.
Ichiro Fukunaga, Yoko Oe, Keiko Danzaki, Sayaka Ohta, Cheng Chen, Madoka Iizumi, Takahiro Shiga, Rina Matsuoka, Takashi Anzai, Remi Hibiya-Motegi, Shori Tajima, Katsuhisa Ikeda, Wado Akamatsu, Kazusaku Kamiya
Stem Cell Research, 2021 53:102290.

【解説書】神谷和作 AAVベクターを用いた内耳への遺伝子治療
神谷和作
実験医学別冊 決定版 ウイルスベクターによる遺伝子導入実験ガイド
Page159-166(2020.11)


Ichiro Fukunaga, Kyoko Shirai, Yoko Oe, Keiko Danzaki, Sayaka Ohta,Takahiro Shiga, Cheng Chen, Katsuhisa Ikeda, Wado Akamatsu, AtsushiKawano, Kazusaku Kamiya
Generation of two induced pluripotent stem cell lines from PBMCs of siblings carrying c.235delC mutation in the GJB2 gene associated with sensorineural hearing loss
Stem Cell Research,2020; 47:101910.

Shori Tajima, Keiko Danzaki, Katsuhisa Ikeda, Kazusaku Kamiya
Degradation and modification of cochlear gap junction proteins in the early development of age-related hearing loss.
Experimental and Molecular Medicine, 2020 ;52(1):166-175.
(日本経済新聞 2020年3月9日 他)


【解説/特集】難聴を治す-2020年版 感音難聴と遺伝子治療
神谷 和作
JOHNS耳鼻咽喉科・頭頸部外科, 2020, 36, 1, 97-100.

Fukunaga I, Shiga T, Chen C, Oe Y, Danzaki K, Ohta S, Matsuoka R, Anzai T, Hibiya-Motegi R, Tajima S, Ikeda K, Akamatsu W, Kamiya K.
Generation of the induced pluripotent stem cell (hiPSC) line (JUFMDOi004-A) from a patient with hearing loss carrying GJB2 (p.V37I) mutation.
Stem Cell Research, 2020, 4;43:101674.

Fukunaga I, Fujimoto A, Hatakeyama K, Kurebayashi N, Ikeda K, Kamiya K.
Generation of Functional CX26-Gap-Junction-Plaque-Forming Cells with Spontaneous Ca2+ Transients via a Gap Junction Characteristic of Developing Cochlea.
Current Protocols in Stem Cell Biology, 2019; 51(1):e100.


Ichiro Fukunaga, Ayumi Fujimoto, Kaori Hatakeyama, Toru Aoki, Atena Nishikawa, Tetsuo Noda, Osamu Minowa, Nagomi Kurebayashi, Katsuhisa Ikeda, Kazusaku Kamiya
In vitro models of GJB2-related hearing loss recapitulate Ca2+ transients via a gap junction characteristic of developing cochlea
Stem Cell Reports, 2016, 7(6), 1023-1036
(読売新聞、米Fox News、英The Sun他)

Takashi Anzai, Ichiro Fukunaga, Kaori Hatakeyama, Ayumi Fujimoto, Kazuma Kobayashi, Atena Nishikawa, Toru Aoki, Tetsuo Noda, Osamu Minowa, Katsuhisa Ikeda, Kazusaku Kamiya. Deformation of the Outer Hair Cells and the Accumulation of Caveolin-2 in Connexin 26 Deficient Mice.
PLoS One, 2015, 10(10):e0141258.

Takashi Iizuka, Kazusaku Kamiya, Satoru Gotoh, Yoshinobu Sugitani, Masaaki Suzuki, Tetsuo Noda, Osamu Minowa, Katsuhisa Ikeda, Perinatal Gjb2 gene transfer rescues hearing in a mouse model of hereditary deafness.
Hum Mol Genet. 2015, 24(13):3651-61.
(朝日新聞、読売新聞、日経新聞、毎日新聞、NHKニュース他)

Kamiya K.
Inner ear cell therapy targeting hereditary deafness by activation of stem cell homing factors.
Frontiers in Pharmacology 2015;6:2.

Kazusaku Kamiya, Vincent Michel, Fabrice Giraudet, Brigitte Riederer, Isabelle Foucher, Samantha Papal, Isabelle Perfettini, Sebastien Le Gal, Elisabeth Verpy, Weiliang Xia, Ursula Seidler, Maria-Magdalena Georgescu, Paul Avan, Aziz El-Amraouia, Christine Petit
An unusually powerful mode of low-frequency sound interference due to outer hair cell hair bundle defects unveiled in Nherf1-/- mice
Proc Natl Acad Sci U S A. 2014, 111(25):9307-12
(科学新聞 他)

Kidokoro Y, Karasawa K, Minowa O, Sugitani Y, Noda T, Ikeda K, Kamiya K.
Deficiency of Transcription Factor Brn4 Disrupts Cochlear Gap Junction Plaques in a Model of DFN3 Non-Syndromic Deafness.
PLoS One. 2014;9(9):e108216. 

Kazusaku Kamiya, Sabrina W. Yum, Nagomi Kurebayashi, Miho Muraki, Kana Ogawa, Keiko Karasawa, Asuka Miwa, Xueshui Guo, Satoru Gotoh, Yoshinobu Sugitani, Hitomi Yamanaka, Shioko Ito-Kawashima, Takashi Iizuka, Takashi Sakurai, Tetsuo Noda, Osamu Minowa, Katsuhisa Ikeda
Assembly of the cochlear gap junction macromolecular complex requires Connexin26
Journal of Clinical Investigation 2014;124(4):1598–1607.
(日経産業新聞、時事通信、ウォールストリートジャーナル他)

Ayako Inoshita, Keiko Karasawa, Megumi Funakubo, Asuka Miwa, Katsuhisa Ikeda, Kazusaku Kamiya
Dominant negative connexin26 mutation R75W causing severe hearing loss influences normal programmed cell death in postnatal organ of Corti
BMC genetics 2014, 15(1):1-8

Nomura N, Kamiya K, Ikeda K, Yui N, Chiga M, Sohara E, Rai T, Sakaki S, Uchida S
Treatment with 17-allylamino-17-demethoxygeldanamycin ameliorated symptoms of Bartter syndrome type IV caused by mutated Bsnd in mice.
Biochem Biophys Res Commun. 2013;441(3):544-9

Homma H, Kamiya K, Kusunoki T, Ikeda K.
Multiplex analyses of cytokine and chemokine release from the cultured fibroblast of nasal polyps: the effect of IL-17A.
Acta Otolaryngol. 2013, 133(10):1065-72.

Gianluca Esposito, Sachine Yoshida, Ryuko Ohnishi, Yousuke Tsuneoka, Maria del Carmen Rostagno, Susumu Yokota, Shota Okabe,
Kazusaku Kamiya, Mikio Hoshino, Masaki Shimizu, Paola Venuti, Takefumi Kikusui, Tadafumi Kato, Kumi O. Kuroda
Infant Calming Responses During Maternal Carrying In Humans and Mice
Current Biology, 2013 23(9):739-45.


Hiroko Okada, Takashi Iizuka, Hideki Mochizuki, Tomoko Nihira, Kazusaku Kamiya, Ayako Inoshita, Hiromi Kasagi, Misato Kasai, Katsuhisa Ikeda, Gene transfer targeting mouse vestibule using adenovirus and adeno-associated virus vectors
Otology & Neurotology, 2012.33(4):655-9

神谷和作 池田勝久
多能性幹細胞を用いた遺伝性難聴に対する内耳細胞治療法の開発
Inner ear cell therapy for hereditary deafness with multipotent stem cells
日本臨床 特集・幹細胞治療 2011 69(12):2215-2219

Yan D, Kamiya K (Co-first), Ouyang XM, Liu XZ.
Analysis of subcellular localization of Myo7a, Pcdh15 and Sans in Ush1c knockout mice.
Int J Exp Pathol. 2011 92(1):66-71.

Hayashi C, Funayama M, Li Y, Kamiya K, Kawano A, Suzuki M, Hattori N, Ikeda K.
Int J Pediatr Otorhinolaryngol. 2011;75(2):211-4.
Prevalence of GJB2 causing recessive profound non-syndromic deafness in Japanese children.

神谷和作、池田勝久 
実験動物を用いた内耳細胞治療研究へのアプローチ 
耳鼻咽喉科臨床 2010 Supple.126:1-5

Kasai M, Hayashi C, Iizuka T, Inoshita A, Kamiya K, Okada H, Nakajima Y, Kaga K, Ikeda K.
Vestibular function of patients with profound deafness related to GJB2 mutation.
Acta Otolaryngol. 2010 Apr 9. [Epub ahead of print]

Fujinami Y, Mutai H, Kamiya K, Mizutari K, Fujii M, Matsunaga T.
Enhanced expression of C/EBP homologous protein (CHOP) precedes degeneration of fibrocytes in the lateral wall after acute cochlear mitochondrial dysfunction induced by 3-nitropropionic acid.
Neurochem Int. 2010 56(3):487-94.

神谷和作 
難聴に対する細胞治療法の開発 
医学のあゆみ 特集号・細胞治療Update, 2009 Vol229, No.9, 863-867

Kazusaku KAMIYA, Cell therapy targeting cochlear fibrocytes, Otology Japan 2009, 19(3):214-218

Minekawa A, Abe T, Inoshita A, Iizuka T, Kakehata S, Narui Y, Koike T, Kamiya K, Okamura HO, Shinkawa H, Ikeda K
Cochlear outer hair cells in a dominant-negative connexin26 mutant mouse preserve non-linear capacitance in spite of impaired distortion product otoacoustic emission, Neuroscience. 2009;164(3):1312-9

Mizutari K, Matsunaga T, Kamiya K, Fujinami, Y Fujii M, Ogawa K
Caspase Inhibitor Facilitates Recovery of Hearing by Protecting the Cochlear Lateral Wall from Acute Cochlear Mitochondrial Dysfunction
Journal of Neuroscience Research, 2008, 86(1):215-22

Ozawa H, Matsunaga T, Kamiya K, Tokumaru Y, Fujii M, Tomita T, Ogawa K
Decreased Expression of Connexin-30 and Aberrant Expression of Connexin-26 in Human Head and Neck Cancer
Anticancer Research, 2007(4B):2189-95

Kamiya K, Fujinami Y, Hoya N, Okamoto Y, Kouike H, Komatsuzaki R, Kusano R, Nakagawa S, Satoh H, Fujii M, Matsunaga T. Mesenchymal stem cell transplantation accelerates hearing recovery through the repair of injured cochlear fibrocytes.
American Journal of Pathology 2007, 171(1):214-26 
(読売新聞他)

Okamoto Y, Hoya N, Kamiya K, Fujii M, Ogawa K, Matsunaga T.
Audiol Neurootology 2005, 20;10(4):220-233

Hoya N, Okamoto Y, Kamiya K, Fujii M, Matsunaga T.
A novel animal model of acute cochlear mitochondrial dysfunction.
Neuroreport 2004, 19;15(10):1597-600

Kamiya K, Kaneda M, Sugawara T, Mazaki E, Okamura N, Montal M, Makita N, Tanaka M, Fukushima K, Fujiwara T, Inoue Y, Yamakawa K.
A nonsense mutation of the sodium channel gene SCN2A in a patient with intractable epilepsy and mental decline.
Journal of Neuroscience 2004, 24(11) 2690-2698
(日経産業新聞、日刊工業新聞、薬事日報、科学新聞他)

Kikkawa Y, Shitara H, Wakana S, Kohara Y, Takada T, Okamoto M, Taya C, Kamiya K, Yoshikawa Y, Tokano H, Kitamura K, Shimizu K, Wakabayashi Y, Shiroishi T, Kominami R, Yonekawa H.
Mutations in a new scaffold protein Sans cause deafness in Jackson shaker mice.
Hum. Mol. Genet. 2003, 12(5). 453-61

Michishita M, Nakamura S, Sakakibara I, Ono F, Fujimoto K, Kamiya K, Ishii Y, Hayashi K, Yoshikawa Y, Takahashi K.
Spontaneous T-cell-rich B-cell lymphoma in a cynomolgus monkey (Macaca fascicularis).
Exp. Anim. 2003, 52(4) 339-44

Kamiya K, Kikkawa Y, Ishii Y, Kyuwa S, Yoshikawa Y.
Changes in mRNA expression in mouse postnatal cochlea by differential display method.
Exp. Anim. 2002, 51(5) 431-435

Kamiya K, Takahashi K, Kitamura K, Momoi T, Yoshikawa Y.
Mitosis and apoptosis in postnatal auditory system.
Brain Res. 2001, 901/1-2, 296-302

Takahashi K, Kamiya K, Urase K, Suga M, Takizawa T, Mori H, Yoshikawa Y, Ichimura K, Kuida K, Momoi T.
Caspase-3-deficiency induces hyperplasia of supporting cells and degeneration of sensory cells resulting in the hearing loss.
Brain Res. 2001, 894, 359-367

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