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Citations to this article

Mechanotransduction in mouse inner ear hair cells requires transmembrane channel–like genes
Yoshiyuki Kawashima, … , Jeffrey R. Holt, Andrew J. Griffith
Yoshiyuki Kawashima, … , Jeffrey R. Holt, Andrew J. Griffith
Published November 21, 2011
Citation Information: J Clin Invest. 2011;121(12):4796-4809. https://doi.org/10.1172/JCI60405.
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Mechanotransduction in mouse inner ear hair cells requires transmembrane channel–like genes

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Abstract

Inner ear hair cells convert the mechanical stimuli of sound, gravity, and head movement into electrical signals. This mechanotransduction process is initiated by opening of cation channels near the tips of hair cell stereocilia. Since the identity of these ion channels is unknown, and mutations in the gene encoding transmembrane channel–like 1 (TMC1) cause hearing loss without vestibular dysfunction in both mice and humans, we investigated the contribution of Tmc1 and the closely related Tmc2 to mechanotransduction in mice. We found that Tmc1 and Tmc2 were expressed in mouse vestibular and cochlear hair cells and that GFP-tagged TMC proteins localized near stereocilia tips. Tmc2 expression was transient in early postnatal mouse cochlear hair cells but persisted in vestibular hair cells. While mice with a targeted deletion of Tmc1 (Tmc1Δ mice) were deaf and those with a deletion of Tmc2 (Tmc2Δ mice) were phenotypically normal, Tmc1ΔTmc2Δ mice had profound vestibular dysfunction, deafness, and structurally normal hair cells that lacked all mechanotransduction activity. Expression of either exogenous TMC1 or TMC2 rescued mechanotransduction in Tmc1ΔTmc2Δ mutant hair cells. Our results indicate that TMC1 and TMC2 are necessary for hair cell mechanotransduction and may be integral components of the mechanotransduction complex. Our data also suggest that persistent TMC2 expression in vestibular hair cells may preserve vestibular function in humans with hearing loss caused by TMC1 mutations.

Authors

Yoshiyuki Kawashima, Gwenaëlle S.G. Géléoc, Kiyoto Kurima, Valentina Labay, Andrea Lelli, Yukako Asai, Tomoko Makishima, Doris K. Wu, Charles C. Della Santina, Jeffrey R. Holt, Andrew J. Griffith

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Nature Communications 2019
Building and repairing the stereocilia cytoskeleton in mammalian auditory hair cells
AC Vélez-Ortega, GI Frolenkov
Hearing Research 2019
Ultrastructural localization of the likely mechanoelectrical transduction channel protein, transmembrane-like channel 1 (TMC1) during development of cochlear hair cells
S Mahendrasingam, DN Furness
Scientific Reports 2019
Targeted Allele Suppression Prevents Progressive Hearing Loss in the Mature Murine Model of Human TMC1 Deafness
H Yoshimura, SB Shibata, PT Ranum, H Moteki, RJ Smith
Molecular Therapy 2019
Subunits of the mechano-electrical transduction channel, Tmc1/2b, require Tmie to localize in zebrafish sensory hair cells
IV Pacentine, T Nicolson, D Raible
PLoS genetics 2019
Stiffness and tension gradients of the hair cell’s tip-link complex in the mammalian cochlea
M Tobin, A Chaiyasitdhi, V Michel, N Michalski, P Martin
eLife 2019
KCNQ1 rescues TMC1 plasma membrane expression but not mechanosensitive channel activity: HARKCOM et al
WT Harkcom, M Papanikolaou, V Kanda, SM Crump, GW Abbott
Journal of Cellular Physiology 2019
Clarin‐2 is essential for hearing by maintaining stereocilia integrity and function
LA Dunbar, P Patni, C Aguilar, P Mburu, L Corns, HR Wells, S Delmaghani, A Parker, S Johnson, D Williams, CT Esapa, MM Simon, L Chessum, S Newton, J Dorning, P Jeyarajan, S Morse, A Lelli, GF Codner, T Peineau, SR Gopal, KN Alagramam, R Hertzano, D Dulon, S Wells, FM Williams, C Petit, SJ Dawson, SD Brown, W Marcotti, A ElAmraoui, MR Bowl
EMBO Molecular Medicine 2019
A Tmc1 mutation reduces calcium permeability and expression of mechanoelectrical transduction channels in cochlear hair cells
M Beurg, A Barlow, DN Furness, R Fettiplace
Proceedings of the National Academy of Sciences 2019
Allele-specific gene editing prevents deafness in a model of dominant progressive hearing loss
B György, C Nist-Lund, B Pan, Y Asai, KD Karavitaki, BP Kleinstiver, SP Garcia, MP Zaborowski, P Solanes, S Spataro, BL Schneider, JK Joung, GS Géléoc, JR Holt, DP Corey
Nature Medicine 2019
An uncharacterized region within the N-terminus of mouse TMC1 precludes trafficking to plasma membrane in a heterologous cell line
DC Soler, M Manikandan, SR Gopal, AE Sloan, TS McCormick, R Stepanyan
Scientific Reports 2019
Hair cell maturation is differentially regulated along the tonotopic axis of the mammalian cochlea
JY Jeng, F Ceriani, A Hendry, SL Johnson, P Yen, DD Simmons, CJ Kros, W Marcotti
The Journal of Physiology 2019
Diversity of cilia-based mechanosensory systems and their functions in marine animal behaviour
LA Bezares-Calderón, J Berger, G Jékely
Philosophical Transactions of the Royal Society B: Biological Sciences 2019
The contribution of TMC1 to adaptation of mechanoelectrical transduction channels in cochlear outer hair cells
AC Goldring, M Beurg, R Fettiplace
The Journal of Physiology 2019
The Development of Cooperative Channels Explains the Maturation of Hair Cell’s Mechanotransduction
F Gianoli, T Risler, AS Kozlov
Biophysical Journal 2019
Distinct functions of TMC channels: a comparative overview
X Yue, Y Sheng, L Kang, R Xiao
Cellular and Molecular Life Sciences 2019
Transient Receptor Potential Cation Channel Subfamily Vanilloid 4 and 3 in the Inner Ear Protect Hearing in Mice
S Wang, Q Geng, L Huo, Y Ma, Y Gao, W Zhang, H Zhang, P Lv, Z Jia
Frontiers in molecular neuroscience 2019
Transcriptomic Analyses of Inner Ear Sensory Epithelia in Zebrafish
Q Yao, L Wang, R Mittal, D Yan, MT Richmond, S Denyer, T Requena, K Liu, GK Varshney, Z Lu, XZ Liu
Anatomical record (Hoboken, N.J. : 2007) 2019
TMC1 is an essential component of a leak channel that modulates tonotopy and excitability of auditory hair cells in mice
S Liu, S Wang, L Zou, J Li, C Song, J Chen, Q Hu, L Liu, P Huang, W Xiong
eLife 2019
Poor maternal nutrition during gestation in sheep alters prenatal muscle growth and development in offspring
MC Gauvin, SM Pillai, SA Reed, JR Stevens, ML Hoffman, AK Jones, SA Zinn, KE Govoni
Journal of animal science 2019
The release of surface-anchored α-tectorin, an apical extracellular matrix protein, mediates tectorial membrane organization
DK Kim, JA Kim, J Park, A Niazi, A Almishaal, S Park
Science Advances 2019
TRPV6, TRPM6 and TRPM7 Do Not Contribute to Hair-Cell Mechanotransduction
CP Morgan, H Zhao, M LeMasurier, W Xiong, B Pan, P Kazmierczak, MR Avenarius, M Bateschell, R Larisch, AJ Ricci, U Müller, PG Barr-Gillespie
Frontiers in cellular neuroscience 2018
Using Drosophila to study mechanisms of hereditary hearing loss
T Li, HJ Bellen, AK Groves
Disease models & mechanisms 2018
Cochlear Gene Therapy for Sensorineural Hearing Loss: Current Status and Major Remaining Hurdles for Translational Success
W Zhang, SM Kim, W Wang, C Cai, Y Feng, W Kong,
Frontiers in molecular neuroscience 2018
The human CIB1–EVER1–EVER2 complex governs keratinocyte-intrinsic immunity to β-papillomaviruses
SJ de Jong, A Créquer, I Matos, D Hum, V Gunasekharan, L Lorenzo, F Jabot-Hanin, E Imahorn, AA Arias, H Vahidnezhad, L Youssefian, JG Markle, E Patin, A DAmico, CQ Wang, F Full, A Ensser, TM Leisner, LV Parise, M Bouaziz, NP Maya, XR Cadena, B Saka, AH Saeidian, N Aghazadeh, S Zeinali, P Itin, JG Krueger, L Laimins, L Abel, E Fuchs, J Uitto, JL Franco, B Burger, G Orth, E Jouanguy, JL Casanova
Journal of Experimental Medicine 2018
Identification of four TMC1 variations in different Chinese families with hereditary hearing loss
H Wang, K Wu, J Guan, J Yang, L Xie, F Xiong, L Lan, D Wang, Q Wang
Molecular Genetics & Genomic Medicine 2018
Mechanically Gated Ion Channels in Mammalian Hair Cells
X Qiu, U Müller
Frontiers in cellular neuroscience 2018
Variable number of TMC1-dependent mechanotransducer channels underlie tonotopic conductance gradients in the cochlea
M Beurg, R Cui, AC Goldring, S Ebrahim, R Fettiplace, B Kachar
Nature Communications 2018
TMC1 Forms the Pore of Mechanosensory Transduction Channels in Vertebrate Inner Ear Hair Cells
B Pan, N Akyuz, XP Liu, Y Asai, C Nist-Lund, K Kurima, BH Derfler, B György, W Limapichat, S Walujkar, LN Wimalasena, M Sotomayor, DP Corey, JR Holt
Neuron 2018
Gene Therapy in Mouse Models of Deafness and Balance Dysfunction
L Wang, JB Kempton, JV Brigande
Frontiers in molecular neuroscience 2018
Structural relationship between the putative hair cell mechanotransduction channel TMC1 and TMEM16 proteins
A Ballesteros, C Fenollar-Ferrer, KJ Swartz
eLife 2018
Eye Movements Evoked by Pulsed Infrared Radiation of the Rat Vestibular System
W Jiang, SM Rajguru
Annals of Biomedical Engineering 2018
Transgenic Tmc2 expression preserves inner ear hair cells and vestibular function in mice lacking Tmc1
Y Asai, B Pan, C Nist-Lund, A Galvin, AN Lukashkin, VA Lukashkina, T Chen, W Zhou, H Zhu, IJ Russell, JR Holt, GS Géléoc
Scientific Reports 2018
TMC Proteins Modulate Egg Laying and Membrane Excitability through a Background Leak Conductance in C. elegans
X Yue, J Zhao, X Li, Y Fan, D Duan, X Zhang, W Zou, Y Sheng, T Zhang, Q Yang, J Luo, S Duan, R Xiao, L Kang
Neuron 2018
On-chip functional neuroimaging with mechanical stimulation in Caenorhabditis elegans larvae for studying development and neural circuits
Y Cho, DN Oakland, SA Lee, WR Schafer, H Lu
Lab on a Chip 2018
Tmc2 expression partially restores auditory function in a mouse model of DFNB7/B11 deafness caused by loss of Tmc1 function
H Nakanishi, K Kurima, B Pan, P Wangemann, TS Fitzgerald, GS Géléoc, JR Holt, AJ Griffith
Scientific Reports 2018
Cell-Specific Transcriptome Analysis Shows That Adult Pillar and Deiters' Cells Express Genes Encoding Machinery for Specializations of Cochlear Hair Cells
H Liu, L Chen, KP Giffen, ST Stringham, Y Li, PD Judge, KW Beisel, DZ He
Frontiers in molecular neuroscience 2018
Transcriptomes of cochlear inner and outer hair cells from adult mice
Y Li, H Liu, KP Giffen, L Chen, KW Beisel, DZ He
Scientific Data 2018
Mechanotransduction is required for establishing and maintaining mature inner hair cells and regulating efferent innervation
LF Corns, SL Johnson, T Roberts, KM Ranatunga, A Hendry, F Ceriani, S Safieddine, KP Steel, A Forge, C Petit, DN Furness, CJ Kros, W Marcotti
Nature Communications 2018
Cryo-EM structure of the mechanically activated ion channel OSCA1.2
S Jojoa-Cruz, K Saotome, SE Murthy, CC Tsui, MS Sansom, A Patapoutian, AB Ward
eLife 2018
Tonotopy in calcium homeostasis and vulnerability of cochlear hair cells
R Fettiplace, JH Nam
Hearing Research 2018
Mouse Magnetic-field Nystagmus in Strong Static Magnetic Fields Is Dependent on the Presence of Nox3:
BK Ward, YH Lee, DC Roberts, E Naylor, AA Migliaccio, CC Santina
Otology & Neurotology 2018
Molecular Structure of the Hair Cell Mechanoelectrical Transduction Complex
CL Cunningham, U Müller
Cold Spring Harbor Perspectives in Medicine 2018
Function and Dysfunction of TMC Channels in Inner Ear Hair Cells
DP Corey, N Akyuz, JR Holt
Cold Spring Harbor Perspectives in Medicine 2018
Molekulares Verstehen des Hörens – Was ändert sich für den Patienten?
T Moser
LARYNGO RHINO OTOL 2018
Cochlear Gene Therapy
L Lustig, O Akil
Cold Spring Harbor Perspectives in Medicine 2018
Identification of ion-channel modulators that protect against aminoglycoside-induced hair-cell death
Emma Kenyon, Nerissa Kirkwood, Sian Kitcher, Molly O'Reilly, Marco Derudas, Daire Cantillon, Richard Goodyear, Abigail Secker, Sarah Baxendale, James Bull, Simon Waddell, Tanya Whitfield, Simon Ward, Corne Kros, Guy Richardson
JCI Insight 2017
Autophagy is essential for hearing in mice
C Fujimoto, S Iwasaki, S Urata, H Morishita, Y Sakamaki, M Fujioka, K Kondo, N Mizushima, T Yamasoba
Cell Death and Disease 2017
Aminoglycoside-Induced Cochleotoxicity: A Review
M Jiang, T Karasawa, PS Steyger
Frontiers in cellular neuroscience 2017
Towards the Prevention of Aminoglycoside-Related Hearing Loss
ME OSullivan, A Perez, R Lin, A Sajjadi, AJ Ricci, AG Cheng
Frontiers in cellular neuroscience 2017
Emerging Gene Therapies for Genetic Hearing Loss
H Ahmed, O Shubina-Oleinik, JR Holt
Journal of the Association for Research in Otolaryngology 2017
Loss of CIB2 Causes Profound Hearing Loss and Abolishes Mechanoelectrical Transduction in Mice
Y Wang, J Li, X Yao, W Li, H Du, M Tang, W Xiong, R Chai, Z Xu
Frontiers in molecular neuroscience 2017
Therapeutic value of nerve growth factor in promoting neural stem cell survival and differentiation and protecting against neuronal hearing loss
Z Han, CP Wang, N Cong, YY Gu, R Ma, FL Chi
Molecular and Cellular Biochemistry 2017
Mechanotransduction current is essential for stability of the transducing stereocilia in mammalian auditory hair cells
AC Vélez-Ortega, MJ Freeman, AA Indzhykulian, JM Grossheim, GI Frolenkov
eLife 2017
Integration of Tmc1/2 into the mechanotransduction complex in zebrafish hair cells is regulated by Transmembrane O-methyltransferase (Tomt)
T Erickson, CP Morgan, J Olt, K Hardy, E Busch-Nentwich, R Maeda, R Clemens, JF Krey, A Nechiporuk, PG Barr-Gillespie, W Marcotti, T Nicolson
eLife 2017
The murine catecholamine methyltransferase mTOMT is essential for mechanotransduction by cochlear hair cells
CL Cunningham, Z Wu, A Jafari, B Zhao, K Schrode, S Harkins-Perry, A Lauer, U Müller
eLife 2017
CIB2 interacts with TMC1 and TMC2 and is essential for mechanotransduction in auditory hair cells
AP Giese, YQ Tang, GP Sinha, MR Bowl, AC Goldring, A Parker, MJ Freeman, SD Brown, S Riazuddin, R Fettiplace, WR Schafer, GI Frolenkov, ZM Ahmed
Nature Communications 2017
d-Tubocurarine and Berbamine: Alkaloids That Are Permeant Blockers of the Hair Cell's Mechano-Electrical Transducer Channel and Protect from Aminoglycoside Toxicity
NK Kirkwood, M O'Reilly, M Derudas, EJ Kenyon, R Huckvale, SM van Netten, SE Ward, GP Richardson, CJ Kros
Frontiers in cellular neuroscience 2017
TMC2 Modifies Permeation Properties of the Mechanoelectrical Transducer Channel in Early Postnatal Mouse Cochlear Outer Hair Cells
LF Corns, JY Jeng, GP Richardson, CJ Kros, W Marcotti
Frontiers in molecular neuroscience 2017
A Model for Link Pruning to Establish Correctly Polarized and Oriented Tip Links in Hair Bundles
N Tompkins, KJ Spinelli, D Choi, PG Barr-Gillespie
Biophysical Journal 2017
PIEZO2 as the anomalous mechanotransducer channel in auditory hair cells: Mechanosensitive channels in cochlear hair cells
M Beurg, R Fettiplace
The Journal of Physiology 2017
Age-dependent gene expression in the inner ear of big brown bats (Eptesicus fuscus)
B Mao, CF Moss, GS Wilkinson, M Smotherman
PloS one 2017
Potential Mechanisms Underlying Inflammation-Enhanced Aminoglycoside-Induced Cochleotoxicity
M Jiang, F Taghizadeh, PS Steyger
Frontiers in cellular neuroscience 2017
A molecular basis for water motion detection by the mechanosensory lateral line of zebrafish
SW Chou, Z Chen, S Zhu, RW Davis, J Hu, L Liu, CA Fernando, K Kindig, WC Brown, R Stepanyan, BM McDermott
Nature Communications 2017
Beyond Cell–Cell Adhesion: Sensational Cadherins for Hearing and Balance
A Jaiganesh, Y Narui, R Araya-Secchi, M Sotomayor
Cold Spring Harbor perspectives in biology 2017
The genetics of hair-cell function in zebrafish
T Nicolson
Journal of Neurogenetics 2017
TMC-1 Mediates Alkaline Sensation in C. elegans through Nociceptive Neurons
X Wang, G Li, J Liu, J Liu, XZ Xu
Neuron 2016
Exome sequencing identifies a mutation in TMC1 as a novel cause of autosomal recessive nonsyndromic hearing loss
J Hu, F Liu, W Xia, L Hao, J Lan, Z Zhu, J Ye, D Ma, Z Ma
Journal of Translational Medicine 2016
Recessive mutations of TMC1 associated with moderate to severe hearing loss
A Imtiaz, A Maqsood, AU Rehman, RJ Morell, JR Holt, TB Friedman, S Naz
neurogenetics 2016
The acquisition of mechano-electrical transducer current adaptation in auditory hair cells requires myosin VI: Hair-cell adaptation requires myosin VI
W Marcotti, LF Corns, RJ Goodyear, AK Rzadzinska, KB Avraham, KP Steel, GP Richardson, CJ Kros
The Journal of Physiology 2016
The Basis of Food Texture Sensation in Drosophila
YV Zhang, TJ Aikin, Z Li, C Montell
Neuron 2016
Transmembrane channel-like ( tmc ) gene regulates Drosophila larval locomotion
Y Guo, Y Wang, W Zhang, S Meltzer, D Zanini, Y Yu, J Li, T Cheng, Z Guo, Q Wang, JS Jacobs, Y Sharma, DF Eberl, MC Göpfert, LY Jan, YN Jan, Z Wang
Proceedings of the National Academy of Sciences 2016
Effects of genetic correction on the differentiation of hair cell-like cells from iPSCs with MYO15A mutation
JR Chen, ZH Tang, J Zheng, HS Shi, J Ding, XD Qian, C Zhang, JL Chen, CC Wang, L Li, JZ Chen, SK Yin, JZ Shao, TS Huang, P Chen, MX Guan, JF Wang
Cell Death and Differentiation 2016
Is TMC1 the Hair Cell Mechanotransducer Channel?
R Fettiplace
Biophysical Journal 2016
Development and localization of reverse-polarity mechanotransducer channels in cochlear hair cells
M Beurg, AC Goldring, AJ Ricci, R Fettiplace
Proceedings of the National Academy of Sciences 2016
RNA Interference Prevents Autosomal-Dominant Hearing Loss
SB Shibata, PT Ranum, H Moteki, B Pan, AT Goodwin, SS Goodman, PJ Abbas, JR Holt, RJ Smith
The American Journal of Human Genetics 2016
Adaptation Independent Modulation of Auditory Hair Cell Mechanotransduction Channel Open Probability Implicates a Role for the Lipid Bilayer
AW Peng, R Gnanasambandam, F Sachs, AJ Ricci
The Journal of neuroscience : the official journal of the Society for Neuroscience 2016
Piezo1 haploinsufficiency does not alter mechanotransduction in mouse cochlear outer hair cells
LF Corns, W Marcotti
Physiological Reports 2016
Tmc1 Point Mutation Affects Ca2+ Sensitivity and Block by Dihydrostreptomycin of the Mechanoelectrical Transducer Current of Mouse Outer Hair Cells
LF Corns, SL Johnson, CJ Kros, W Marcotti
The Journal of neuroscience : the official journal of the Society for Neuroscience 2016
Natural Compounds as Occult Ototoxins? Ginkgo biloba Flavonoids Moderately Damage Lateral Line Hair Cells
S Neveux, NK Smith, A Roche, BE Blough, W Pathmasiri, AB Coffin
Journal of the Association for Research in Otolaryngology 2016
Mechanotransduction as an Adaptation to Gravity
T Najrana, J Sanchez-Esteban
Frontiers in Pediatrics 2016
Mechanosensory hair cells express two molecularly distinct mechanotransduction channels
Z Wu, N Grillet, B Zhao, C Cunningham, S Harkins-Perry, B Coste, S Ranade, N Zebarjadi, M Beurg, R Fettiplace, A Patapoutian, U Müller
Nature Neuroscience 2016
Morin hydrate promotes inner ear neural stem cell survival and differentiation and protects cochlea against neuronal hearing loss
Q He, Z Jia, Y Zhang, X Ren
Journal of Cellular and Molecular Medicine 2016
Connexin-Mediated Signaling in Nonsensory Cells Is Crucial for the Development of Sensory Inner Hair Cells in the Mouse Cochlea
SL Johnson, F Ceriani, O Houston, R Polishchuk, E Polishchuk, G Crispino, V Zorzi, F Mammano, W Marcotti
The Journal of neuroscience : the official journal of the Society for Neuroscience 2016
Molecular Identity of the Mechanotransduction Channel in Hair Cells: Not Quiet There Yet
Z Wu, U Muller
The Journal of neuroscience : the official journal of the Society for Neuroscience 2016
Are TMCs the Mechanotransduction Channels of Vertebrate Hair Cells?
DP Corey, JR Holt
The Journal of neuroscience : the official journal of the Society for Neuroscience 2016
Distinct capacity for differentiation to inner ear cell types by progenitor cells of the cochlea and vestibular organs
WJ McLean, DT McLean, RA Eatock, AS Edge
Development (Cambridge, England) 2016
Designer aminoglycosides prevent cochlear hair cell loss and hearing loss
Markus Huth, Kyu-Hee Han, Kayvon Sotoudeh, Yi-Ju Hsieh, Thomas Effertz, Andrew Vu, Sarah Verhoeven, Michael Hsieh, Robert Greenhouse, Alan Cheng, Anthony J Ricci
Journal of Clinical Investigation 2015
Unconventional Mechanics of Lipid Membranes: A Potential Role for Mechanotransduction of Hair Cell Stereocilia
J Kim
Biophysical Journal 2015
A TMC1 (transmembrane channel-like 1) mutation (p.S320R) in a Polish family with hearing impairment
MA Hassan, AA Shah, E Szmida, R Smigiel, MM Sasiadek, M Pfister, N Blin, A Bress
Journal of Applied Genetics 2015
Deficiency of Angulin-2/ILDR1, a Tricellular Tight Junction-Associated Membrane Protein, Causes Deafness with Cochlear Hair Cell Degeneration in Mice
T Higashi, T Katsuno, S Kitajiri, M Furuse, M Koval
PloS one 2015
Local mechanisms for loud sound-enhanced aminoglycoside entry into outer hair cells
H Li, A Kachelmeier, DN Furness, PS Steyger
Frontiers in cellular neuroscience 2015
Gene Expression by Mouse Inner Ear Hair Cells during Development
DI Scheffer, J Shen, DP Corey, ZY Chen
The Journal of neuroscience : the official journal of the Society for Neuroscience 2015
Characterization of the Transcriptome of Nascent Hair Cells and Identification of Direct Targets of the Atoh1 Transcription Factor
T Cai, HI Jen, H Kang, TJ Klisch, HY Zoghbi, AK Groves
The Journal of neuroscience : the official journal of the Society for Neuroscience 2015
The molecules that mediate sensory transduction in the mammalian inner ear
B Pan, JR Holt
Current Opinion in Neurobiology 2015
Tmc gene therapy restores auditory function in deaf mice
C Askew, C Rochat, B Pan, Y Asai, H Ahmed, E Child, BL Schneider, P Aebischer, JR Holt
Science Translational Medicine 2015
Targeted gene capture and massively parallel sequencing identify TMC1 as the causative gene in a six-generation Chinese family with autosomal dominant hearing loss
X Gao, SS Huang, YY Yuan, GJ Wang, JC Xu, YB Ji, MY Han, F Yu, DY Kang, X Lin, P Dai
American Journal of Medical Genetics Part A 2015
Green Tea Polyphenols Protects Cochlear Hair Cells from Ototoxicity by Inhibiting Notch Signalling
LT Gu, J Yang, SZ Su, WW Liu, ZG Shi, QR Wang
Neurochemical Research 2015
Cellular signaling protective against noise-induced hearing loss – A role for novel intrinsic cochlear signaling involving corticotropin-releasing factor?
DE Vetter
Biochemical Pharmacology 2015
TMC1 and TMC2 Localize at the Site of Mechanotransduction in Mammalian Inner Ear Hair Cell Stereocilia
K Kurima, S Ebrahim, B Pan, M Sedlacek, P Sengupta, BA Millis, R Cui, H Nakanishi, T Fujikawa, Y Kawashima, BY Choi, K Monahan, JR Holt, AJ Griffith, B Kachar
Cell Reports 2015
The effects of Tmc1 Beethoven mutation on mechanotransducer channel function in cochlear hair cells
M Beurg, AC Goldring, R Fettiplace
The Journal of General Physiology 2015
NaHS Protects Cochlear Hair Cells from Gentamicin-Induced Ototoxicity by Inhibiting the Mitochondrial Apoptosis Pathway
Y Dong, D Liu, Y Hu, X Ma, B Alsina
PloS one 2015
The elusive mechanotransduction machinery of hair cells
B Zhao, U Müller
Current Opinion in Neurobiology 2015
The 133-kDa N-terminal domain enables myosin 15 to maintain mechanotransducing stereocilia and is essential for hearing: ( A ) Two protein isoforms are generated from alternatively spliced transcripts of Myo15 . Transcripts incorporating exon 2 encode isoform 1 (396 kDa; Genbank: NM_010862.2), while exclusion of this exon produces isoform 2 (262 kDa; Genbank: NM_182698.2). Both isoforms have identical motor and tail domains, including a PDZ ligand, SH3, MyTH4 and FERM moieties. The mutant Myo15 alleles used in this study are shown along with antibody epitopes. ( B ) Auditory brainstem response (ABR) thresholds at 4, 20 and 48 kHz for Myo15 +/+ , Myo15+/ΔN and Myo15ΔN/ΔN mice at 6 weeks of age. Data are mean ± SD (n = 3–6 animals per group). ( C ) ABR thresholds at 20 kHz, measured from 2, 4 and 6 weeks old Myo15+/+, Myo15+/ΔN and Myo15ΔN/ΔN mice. Data are mean ± SD (n = 3–6 animals per group). ( D ) Distortion product otoacoustic emission (DPOAE) levels (2F1-F2) at 12 kHz in Myo15+/+ and Myo15ΔN/ΔN mice at 6 weeks. Data are mean ± SD (n = 3–4 animals per group). ( E – G ) Relative expression of Myo15 isoforms in wild-type cochleae measured with RT-qPCR at ages indicated. Probes target exon junction 13–14, detecting the motor domain common to both isoforms 1 and 2 ( E ); exon junction 2–3, detecting isoform 1 ( F ); exon junction 1–3, detecting isoform 2 ( G ). Relative expression 2^(-∆∆C T ) for each Myo15 transcript was normalized first to the housekeeping gene ( Tbp ) and then to the respective isoform expression at P0. The total expression of both isoforms remains stable ( E ), however there is a transition from isoform 2 to isoform 1, which becomes the dominant mRNA species by P21 (see Figure 1—figure supplement 1D). Data are mean ± SD (n = 3–5 biological replicates per condition). Asterisks indicate significance: n.s., p > 0.05; **, p < 0.01; ****, p < 0.0001 (ANOVA with Tukey's multiple comparison test). ( H ) Identical qPCR probes were used to assay Myo15 expression in Myo15+/ΔN and Myo15ΔN/ΔN cochleae at P0. Relative expression 2^(-∆∆C T ) values were normalized to Tbp and then to expression in heterozygous Myo15+/∆N samples. Data are mean ± SD (n = 3–4 biological replicates per condition). n.s., p > 0.05 ( t -test of independent variables)
Q Fang, AA Indzhykulian, M Mustapha, GP Riordan, DF Dolan, TB Friedman, IA Belyantseva, GI Frolenkov, SA Camper, JE Bird
eLife 2015
Mechanically Activated Ion Channels
SS Ranade, R Syeda, A Patapoutian
Neuron 2015
A Novel C-Terminal CIB2 (Calcium and Integrin Binding Protein 2) Mutation Associated with Non-Syndromic Hearing Loss in a Hispanic Family
K Patel, AP Giese, JM Grossheim, RS Hegde, M Delio, J Samanich, S Riazuddin, GI Frolenkov, J Cai, ZM Ahmed, BE Morrow, KW Koch
PloS one 2015
Modulating Ca2+ signals: a common theme for TMEM16, Ist2, and TMC
K Kunzelmann, I Cabrita, P Wanitchakool, J Ousingsawat, L Sirianant, R Benedetto, R Schreiber
Pflügers Archiv - European Journal of Physiology 2015
Genetics of auditory mechano-electrical transduction
N Michalski, C Petit
Pflügers Archiv - European Journal of Physiology 2014
TMC function in hair cell transduction
JR Holt, B Pan, MA Koussa, Y Asai
Hearing Research 2014
Conductance and block of hair-cell mechanotransducer channels in transmembrane channel-like protein mutants
M Beurg, KX Kim, R Fettiplace
The Journal of General Physiology 2014
Mutations of TMC1 cause deafness by disrupting mechanoelectrical transduction
H Nakanishi, K Kurima, Y Kawashima, AJ Griffith
Auris, Nasus, Larynx 2014
Whole exome sequencing identifies new causative mutations in tunisian families with non-syndromic deafness
Z Riahi, C Bonnet, R Zainine, M Louha, Y Bouyacoub, N Laroussi, M Chargui, R Kefi, L Jonard, I Dorboz, JP Hardelin, SB Salah, J Levilliers, D Weil, K McElreavey, OT Boespflug, G Besbes, S Abdelhak, C Petit
PloS one 2014
A novel DFNA36 mutation in TMC1 orthologous to the Beethoven (Bth) mouse associated with autosomal dominant hearing loss in a Chinese family
Y Zhao, D Wang, L Zong, F Zhao, L Guan, P Zhang, W Shi, L Lan, H Wang, Q Li, B Han, L Yang, X Jin, J Wang, J Wang, Q Wang
PloS one 2014
Transduction without tip links in cochlear hair cells is mediated by ion channels with permeation properties distinct from those of the mechano-electrical transducer channel
W Marcotti, LF Corns, T Desmonds, NK Kirkwood, GP Richardson, CJ Kros
The Journal of neuroscience : the official journal of the Society for Neuroscience 2014
Transmembrane channel-like (TMC) genes are required for auditory and vestibular mechanosensation
Y Kawashima, K Kurima, B Pan, AJ Griffith, JR Holt
Pflügers Archiv - European Journal of Physiology 2014
Using injectoporation to deliver genes to mechanosensory hair cells
W Xiong, T Wagner, L Yan, N Grillet, U Müller
Nature Protocols 2014
Tip-link protein protocadherin 15 interacts with transmembrane channel-like proteins TMC1 and TMC2
R Maeda, KS Kindt, W Mo, CP Morgan, T Erickson, H Zhao, R Clemens-Grisham, PG Barr-Gillespie, T Nicolson
Proceedings of the National Academy of Sciences 2014
Mechanosensory molecules and circuits in C. elegans
WR Schafer
Pflügers Archiv - European Journal of Physiology 2014
Sound strategies for hearing restoration
GS Géléoc, JR Holt
Science 2014
Calcium entry into stereocilia drives adaptation of the mechanoelectrical transducer current of mammalian cochlear hair cells
LF Corns, SL Johnson, CJ Kros, W Marcotti
Proceedings of the National Academy of Sciences 2014
TMIE Is an Essential Component of the Mechanotransduction Machinery of Cochlear Hair Cells
B Zhao, Z Wu, N Grillet, L Yan, W Xiong, S Harkins-Perry, U Müller
Neuron 2014
Subunit determination of the conductance of hair-cell mechanotransducer channels
M Beurg, W Xiong, B Zhao, U Müller, R Fettiplace
Proceedings of the National Academy of Sciences 2014
Characterization of Transcriptomes of Cochlear Inner and Outer Hair Cells
H Liu, JL Pecka, Q Zhang, GA Soukup, KW Beisel, DZ He
The Journal of neuroscience : the official journal of the Society for Neuroscience 2014
The how and why of identifying the hair cell mechano-electrical transduction channel
T Effertz, AL Scharr, AJ Ricci
Pflügers Archiv - European Journal of Physiology 2014
The Physiology of Mechanoelectrical Transduction Channels in Hearing
R Fettiplace, KX Kim
Physiological reviews 2014
Deletion of PDZD7 disrupts the Usher syndrome type 2 protein complex in cochlear hair cells and causes hearing loss in mice
J Zou, T Zheng, C Ren, C Askew, XP Liu, B Pan, JR Holt, Y Wang, J Yang
Human Molecular Genetics 2013
TMC1 and TMC2 Are Components of the Mechanotransduction Channel in Hair Cells of the Mammalian Inner Ear
B Pan, GS Géléoc, Y Asai, GC Horwitz, K Kurima, K Ishikawa, Y Kawashima, AJ Griffith, JR Holt
Neuron 2013
tmc-1 encodes a sodium-sensitive channel required for salt chemosensation in C. elegans
M Chatzigeorgiou, S Bang, SW Hwang, WR Schafer
Nature 2013
Permeation of Fluorophore-Conjugated Phalloidin into Live Hair Cells of the Inner Ear Is Modulated by P2Y Receptors
BR Thiede, JT Corwin
Journal of the Association for Research in Otolaryngology 2013
Who needs tip links? Backwards transduction by hair cells
PG Barr-Gillespie, T Nicolson
The Journal of General Physiology 2013
The role of transmembrane channel-like proteins in the operation of hair cell mechanotransducer channels
KX Kim, M Beurg, CM Hackney, DN Furness, S Mahendrasingam, R Fettiplace
The Journal of General Physiology 2013
Transient receptor potential melastatin 1: a hair cell transduction channel candidate
J Gerka-Stuyt, A Au, NS Peachey, KN Alagramam
PloS one 2013
The evolutionary and genetic origins of consciousness in the Cambrian Period over 500 million years ago
TE Feinberg, J Mallatt
Frontiers in psychology 2013
Notch signaling limits supporting cell plasticity in the hair cell-damaged early postnatal murine cochlea
S Korrapati, I Roux, E Glowatzki, A Doetzlhofer
PloS one 2013
Molecular remodeling of tip links underlies mechanosensory regeneration in auditory hair cells
AA Indzhykulian, R Stepanyan, A Nelina, KJ Spinelli, ZM Ahmed, IA Belyantseva, TB Friedman, PG Barr-Gillespie, GI Frolenkov
PLoS Biology 2013
Integrity and regeneration of mechanotransduction machinery regulate aminoglycoside entry and sensory cell death
AA Vu, GS Nadaraja, ME Huth, L Luk, J Kim, R Chai, AJ Ricci, AG Cheng
PloS one 2013
Usher proteins in inner ear structure and function
ZM Ahmed, GI Frolenkov, S Riazuddin
Physiological genomics 2013
Salty sensations
B Coste, A Patapoutian
Nature 2013
Genetics of Hearing and Deafness
S Angeli, X Lin, XZ Liu
The Anatomical Record: Advances in Integrative Anatomy and Evolutionary Biology 2012
TRPC3 and TRPC6 are essential for normal mechanotransduction in subsets of sensory neurons and cochlear hair cells
K Quick, J Zhao, N Eijkelkamp, JE Linley, F Rugiero, JJ Cox, R Raouf, M Gringhuis, JE Sexton, J Abramowitz, R Taylor, A Forge, J Ashmore, N Kirkwood, CJ Kros, GP Richardson, M Freichel, V Flockerzi, L Birnbaumer, JN Wood
Open Biology 2012
How We Feel: Ion Channel Partnerships that Detect Mechanical Inputs and Give Rise to Touch and Pain Perception
SL Geffeney, MB Goodman
Neuron 2012
Gentamicin Reduces Calcific Nodule Formation by Aortic Valve Interstitial Cells In Vitro
A Kumar, DC Wiltz, KJ Grande-Allen
Cardiovascular Engineering and Technology 2012
TMHS Is an Integral Component of the Mechanotransduction Machinery of Cochlear Hair Cells
W Xiong, N Grillet, HM Elledge, TF Wagner, B Zhao, KR Johnson, P Kazmierczak, U Müller
Cell 2012
Developmental changes in the cochlear hair cell mechanotransducer channel and their regulation by transmembrane channel-like proteins
KX Kim, R Fettiplace
The Journal of General Physiology 2012
Dendritic Filopodia, Ripped Pocket, NOMPC, and NMDARs Contribute to the Sense of Touch in Drosophila Larvae
A Tsubouchi, JC Caldwell, WD Tracey
Current Biology 2012
Loss of Slc4a1b chloride/bicarbonate exchanger function protects mechanosensory hair cells from aminoglycoside damage in the zebrafish mutant persephone
DW Hailey, B Roberts, KN Owens, AK Stewart, T Linbo, R Pujol, SL Alper, EW Rubel, DW Raible
PLoS genetics 2012
HCN1 and HCN2 proteins are expressed in cochlear hair cells: HCN1 can form a ternary complex with protocadherin 15 CD3 and F-actin-binding filamin A or can interact with HCN2
NA Ramakrishnan, MJ Drescher, KM Khan, JS Hatfield, DG Drescher
The Journal of biological chemistry 2012
Permeation properties of the hair cell mechanotransducer channel provide insight into its molecular structure
B Pan, J Waguespack, ME Schnee, C LeBlanc, AJ Ricci
Journal of neurophysiology 2012
Touch sense
Y Roudaut, A Lonigro, B Coste, J Hao, P Delmas, M Crest
Channels (Austin, Tex.) 2012
A perception of sound and gravity by TMC1 and TMC2
Erick Lin
Journal of Clinical Investigation 2011
Comprehensive Physiology
R Terjung
Comprehensive Physiology 2011
Pulmonary surfactant: an immunological perspective
ZC Chroneos, Z Sever-Chroneos, VL Shepherd
Cellular physiology and biochemistry : international journal of experimental cellular physiology, biochemistry, and pharmacology 2009

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