(Department of Otorhinolaryngology, Juntendo University Faculty of Medicine)

Our group aims to discover the molecular mechanisms of hereditary deafness especially for GJB2 associated deafness which is the most typical type in congenital hearing loss. Our goal is to develop novel strategies for inner ear stem cell therapy with multipotent stem cell such as induced pluripotent stem (iPS) cell, gene therapy with virus vector such as adeno associated virus (AAV) and drag screening. These strategies are expected to cure the hearing loss of those patients in near future.

Lab Members

Associate Professor Kazusaku Kamiya, Ph.D.
Postdoctoral Fellow Ichiro Fukunaga, Ph.D.
Postdoctoral Fellow Ayumi Fujimoto, D.V.M., Ph.D.
Research Assistant Kaori Hatakeyama
Research Assistant Atena Nishikawa
Research Assistant Cheng Chen, Ph.D.

Research article

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 (Published Online: November 10, 2016)
DOI: http://dx.doi.org/10.1016/j.stemcr.2016.10.005

Kazusaku Kamiya, Ichiro Fukunaga, Kaori Hatakeyama, Katsuhisa Ikeda
Connexin26 regulates assembly and maintenance of cochlear gap junction macromolecular complex for normal hearing
AIP Conf. Proc. 1703, 030018 (2015); http://dx.doi.org/10.1063/1.4939333

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.

Kamiya K, Karasawa K, Kobayashi K, Miwa A, Ikeda K
Differentiation of iPS Cells to Cochlear Cells are Regulated Depending on the Part of Co-cultured Organs.
J Otol Rhinol, 2015, S1:1. doi:http://dx.doi.org/10.4172/2324-8785.S1-008

Okada H, Kamiya K, Iizuka T, Ikeda K
Postnatal Development and Maturation of the Vestibular Organ in Dominant-Negative Connexin 26 Transgenic Mouse.
J Otol Rhinol, 2015, S1:1. doi:http://dx.doi.org/10.4172/2324-8785.S1-009

Iizuka T, Kamiya K, Gotoh S, Sugitani Y, Suzuki M, Noda T, Minowa O, Ikeda K.
Perinatal Gjb2 gene transfer rescues hearing in a mouse model of hereditary deafness.
Hum Mol Genet. 2015, 24(13):3651-61.

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

Yoshinobu Kidokoro, Keiko Karasawa, Osamu Minowa, Yoshinobu Sugitani, Tetsuo Noda, Katsuhisa Ikeda, Kazusaku Kamiya
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, 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.

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.

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

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.

Cell therapy for hereditary hearing loss with stem cell homing factors.
Nihon Yakurigaku Zasshi. 2013;141(4):191-4.

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

Kazusaku Kamiya, Katsuhisa Ikeda
Inner ear cell therapy for hereditary deafness with multipotent stem cells
Nihon Rinsho. 2011 (12):2215-9.

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

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. 

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 130(9):990-5.

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.

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

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

News Release

Sunday Express (UK) 20/11/2016
“Stem Cells could be used to cure deafness from birth, scientists say”
http://www.express.co.uk/news/science/734203/Stem-cells-used-cure-congenital-deafness-birth-scientists-research

Fox News (USA) 21/11/2016
“Stem cells could cure hereditary deafness within 5 year”
http://www.foxnews.com/health/2016/11/21/stem-cells-could-cure-hereditary-deafness-within-5-years.html

The Sun (UK) 20/11/2016
“Scientists ‘on the brink of a cure for deafness’ with stem cells to correct hereditary defect”
https://www.thesun.co.uk/news/2225769/scientists-on-the-brink-of-a-cure-for-deafness-with-stem-cells-to-correct-hereditary-defect/

Daily Mail (UK) 21/11/2016
“Can deafness be cured using stem cells? Treatment the can allow people to hear again may be ready in 10 years”
http://www.dailymail.co.uk/sciencetech/article-3956674/Can-deafness-cured-using-stem-cells-Treatment-allow-people-hear-ready-10-years.html

Daily Telegraph (UK) 21/11/2016
“Scientists ‘on the brink of a cure for deafness’ with stem cells to correct hereditary defect”
http://www.dailytelegraph.com.au/lifestyle/health/scientists-on-the-brink-of-a-cure-for-deafness-with-stem-cells-to-correct-hereditary-defect/news-story/82bdae431890b1952512461f59a596b3

Daily Mirror (UK) 20/11/2016
“Scientists believe they have found a cure for deafness by replacing faulty cells in the human ear”
http://www.mirror.co.uk/science/scientists-believe-found-cure-deafness-9298845

News.com (Australia) 22/11/2016
“Scientists ‘on the brink of a cure for deafness’ with stem cells to correct hereditary defect”
http://www.news.com.au/lifestyle/health/health-problems/scientists-on-the-brink-of-a-cure-for-deafness-with-stem-cells-to-correct-hereditary-defect/news-story/82bdae431890b1952512461f59a596b3

Gènéthique (France) 22/11/2016
“Les cellules souches seraient susceptibles de soigner une surdité congénitale d’ici 10 ans”
http://genethique.org/fr/les-cellules-souches-seraient-susceptibles-de-soigner-une-surdite-congenitale-dici-10-ans-66564.html#.WE9AQ4VOIyY

PRESS RELEASE: Juntendo University 11/11/2016
Stem cells provide sound in vitro models for deafness
http://www.juntendo.ac.jp/albums/abm.php?f=abm00018060.pdf&n=Juntendo+Press+Release+20161111.pdf

E-mail
kkamiya[@]juntendo.ac.jp