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TMC1 and TMC2 Localize at the Site of Mechanotransduction in Mammalian Inner Ear Hair Cell Stereocilia.

Kurima K, Ebrahim S, Pan B, Sedlacek M, Sengupta P, Millis BA, Cui R, Nakanishi H, Fujikawa T, Kawashima Y, Choi BY, Monahan K, Holt JR, Griffith AJ, Kachar B.

Cell Rep. 2015 Aug 26. pii: S2211-1247(15)00848-7. doi: 10.1016/j.celrep.2015.07.058. [Epub ahead of print]


The molecules that mediate sensory transduction in the mammalian inner ear.

Pan B, Holt JR.

Curr Opin Neurobiol. 2015 Jul 24;34:165-171. doi: 10.1016/j.conb.2015.06.013. [Epub ahead of print] Review.


Tmc gene therapy restores auditory function in deaf mice.

Askew C, Rochat C, Pan B, Asai Y, Ahmed H, Child E, Schneider BL, Aebischer P, Holt JR.

Sci Transl Med. 2015 Jul 8;7(295):295ra108. doi: 10.1126/scitranslmed.aab1996.


Transmembrane channel-like (TMC) genes are required for auditory and vestibular mechanosensation.

Kawashima Y, Kurima K, Pan B, Griffith AJ, Holt JR.

Pflugers Arch. 2015 Jan;467(1):85-94. doi: 10.1007/s00424-014-1582-3. Epub 2014 Jul 31.


Strain imaging of nanoscale semiconductor heterostructures with x-ray Bragg projection ptychography.

Holt MV, Hruszkewycz SO, Murray CE, Holt JR, Paskiewicz DM, Fuoss PH.

Phys Rev Lett. 2014 Apr 25;112(16):165502. Epub 2014 Apr 23.


Sound strategies for hearing restoration.

Géléoc GS, Holt JR.

Science. 2014 May 9;344(6184):1241062. doi: 10.1126/science.1241062. Review.


Mechanotransduction and hyperpolarization-activated currents contribute to spontaneous activity in mouse vestibular ganglion neurons.

Horwitz GC, Risner-Janiczek JR, Holt JR.

J Gen Physiol. 2014 Apr;143(4):481-97. doi: 10.1085/jgp.201311126. Epub 2014 Mar 17.


TMC function in hair cell transduction.

Holt JR, Pan B, Koussa MA, Asai Y.

Hear Res. 2014 May;311:17-24. doi: 10.1016/j.heares.2014.01.001. Epub 2014 Jan 11. Review.


Deletion of PDZD7 disrupts the Usher syndrome type 2 protein complex in cochlear hair cells and causes hearing loss in mice.

Zou J, Zheng T, Ren C, Askew C, Liu XP, Pan B, Holt JR, Wang Y, Yang J.

Hum Mol Genet. 2014 May 1;23(9):2374-90. doi: 10.1093/hmg/ddt629. Epub 2013 Dec 11.


A Gata3-Mafb transcriptional network directs post-synaptic differentiation in synapses specialized for hearing.

Yu WM, Appler JM, Kim YH, Nishitani AM, Holt JR, Goodrich LV.

Elife. 2013 Dec 10;2:e01341. doi: 10.7554/eLife.01341.


Functional contributions of HCN channels in the primary auditory neurons of the mouse inner ear.

Kim YH, Holt JR.

J Gen Physiol. 2013 Sep;142(3):207-23. doi: 10.1085/jgp.201311019.


TMC1 and TMC2 are components of the mechanotransduction channel in hair cells of the mammalian inner ear.

Pan B, Géléoc GS, Asai Y, Horwitz GC, Kurima K, Ishikawa K, Kawashima Y, Griffith AJ, Holt JR.

Neuron. 2013 Aug 7;79(3):504-15. doi: 10.1016/j.neuron.2013.06.019. Epub 2013 Jul 18.


Gene therapy for deaf mice goes viral.

Holt JR, Vandenberghe LH.

Mol Ther. 2012 Oct;20(10):1836-7. doi: 10.1038/mt.2012.196. No abstract available.


The mechanosensory structure of the hair cell requires clarin-1, a protein encoded by Usher syndrome III causative gene.

Geng R, Melki S, Chen DH, Tian G, Furness DN, Oshima-Takago T, Neef J, Moser T, Askew C, Horwitz G, Holt JR, Imanishi Y, Alagramam KN.

J Neurosci. 2012 Jul 11;32(28):9485-98. doi: 10.1523/JNEUROSCI.0311-12.2012.


The function and molecular identity of inward rectifier channels in vestibular hair cells of the mouse inner ear.

Levin ME, Holt JR.

J Neurophysiol. 2012 Jul;108(1):175-86. doi: 10.1152/jn.00098.2012. Epub 2012 Apr 11.


Mechanotransduction in mouse inner ear hair cells requires transmembrane channel-like genes.

Kawashima Y, Géléoc GS, Kurima K, Labay V, Lelli A, Asai Y, Makishima T, Wu DK, Della Santina CC, Holt JR, Griffith AJ.

J Clin Invest. 2011 Dec;121(12):4796-809. doi: 10.1172/JCI60405. Epub 2011 Nov 21.


HCN channels expressed in the inner ear are necessary for normal balance function.

Horwitz GC, Risner-Janiczek JR, Jones SM, Holt JR.

J Neurosci. 2011 Nov 16;31(46):16814-25. doi: 10.1523/JNEUROSCI.3064-11.2011.


Polycystin-1 is required for stereocilia structure but not for mechanotransduction in inner ear hair cells.

Steigelman KA, Lelli A, Wu X, Gao J, Lin S, Piontek K, Wodarczyk C, Boletta A, Kim H, Qian F, Germino G, Géléoc GS, Holt JR, Zuo J.

J Neurosci. 2011 Aug 24;31(34):12241-50. doi: 10.1523/JNEUROSCI.6531-10.2011.


Gipc3 mutations associated with audiogenic seizures and sensorineural hearing loss in mouse and human.

Charizopoulou N, Lelli A, Schraders M, Ray K, Hildebrand MS, Ramesh A, Srisailapathy CR, Oostrik J, Admiraal RJ, Neely HR, Latoche JR, Smith RJ, Northup JK, Kremer H, Holt JR, Noben-Trauth K.

Nat Commun. 2011 Feb 15;2:201. doi: 10.1038/ncomms1200.


Development and regeneration of sensory transduction in auditory hair cells requires functional interaction between cadherin-23 and protocadherin-15.

Lelli A, Kazmierczak P, Kawashima Y, Müller U, Holt JR.

J Neurosci. 2010 Aug 25;30(34):11259-69. doi: 10.1523/JNEUROSCI.1949-10.2010.

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