Publications

For a full list of Holt/Geleoc Lab publications, please click on this link: 
PubMed Holt/Geleoc Lab

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Efficient Viral Transduction in Fetal and Adult Human Inner Ear Explants with AAV9-PHP.B Vectors

Edward S. A. van Beelen, Wouter H. van der Valk, Thijs O. Verhagen, John C. M. J. de Groot, Margot A. Madison, Wijs Shadmanfar, 

Erik F. Hensen, Jeroen C. Jansen, Peter Paul G. van Benthem, Jeffrey R. Holt* and Heiko Locher*  *Co-senior authors

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June, 2022

Optimized AAV Vectors for TMC1 Gene Therapy in a Humanized Mouse Model of DFNB7/11

Irina Marcovich,  Nicholas K. Baer, Olga Shubina-Oleinik, Rachel Eclov, Clayton W. Beard and Jeffrey R. Holt

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Link to Biomolecules
Link to Biomolecules

March 25, 2022

pH regulates potassium conductance and drives a constitutive proton current in human TMEM

Wang Zheng, Chen Shen, Longfei Wang, Shaun Rawson, Wen Jun Xie, Carl Nist-Lund, Jason Wu, Zhangfei Shen , Shiyu Xia, Jeffrey R Holt*, Hao Wu*, Tian-Min Fu*   *Co-senior authors

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Link to Science Advances Article

December 15, 2021

Dual-vector gene therapy restores auditory sensitivity in a mouse model of DFNB16 hearing loss

Olga Shubina-Oleinik, Carl Nist-Lund, Courtney French, Shira Rockowitz, A Eliot Shearer, Jeffrey R Holt

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Link to Science Advances Article

November 4, 2021

Sensory transduction is required for normal development and maturation of cochlear inner hair cell synapses

John Lee,  Kosuke Kawai, Jeffrey R Holt, Gwenaëlle SG Géléoc

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Link to eLife Article

November 4, 2021

Putting the Pieces Together: the Hair Cell Transduction Complex

Jeffrey R Holt, Mélanie Tobin, Johannes Elferich, Eric Gouaux, Angela Ballesteros, Zhiqiang Yan, Zubair M Ahmed, Teresa Nicolson

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Link to JARO Article

February 5, 2021

Neonatal AAV gene therapy rescues hearing in a mouse model of SYNE4 deafness

Shahar Taiber,  Roie Cohen,  Ofer Yizhar‐Barnea, David Sprinzak, Jeffrey R Holt, Karen B Avraham

Link to EMBO Article

December 2, 2020

Direct Delivery of Antisense Oligonucleotides to the Middle and Inner Ear Improves Hearing and Balance in Usher Mice

Jennifer J Lentz , Bifeng Pan , Abhilash Ponnath , Christopher M Tran , Carl Nist-Lund , Alice Galvin , Hannah Goldberg , Katelyn N Robillard , Francine M Jodelka , Hamilton E Farris , Jun Huang , Tianwen Chen , Hong Zhu , Wu Zhou , Frank Rigo , Michelle L Hastings , Gwenaëlle S G Géléoc

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Link to Molecular Therapy

November 17, 2020

Single and Dual Vector Gene Therapy with AAV9-PHP.B Rescues Hearing in Tmc1 Mutant Mice.  Wu J, Solanes P, Nist-Lund C, Spataro S, Shubina-Oleinik O, Marcovich I, Goldberg H, Schneider BL, Holt JR. 

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Link to Molecular Therapy

June 03, 2020

In vivo base editing restores sensory transduction and transiently improves auditory function in a mouse model of recessive deafness

Wei-Hsi Yeh*, Olga Shubina-Oleinik*, Jonathan M. Levy, Bifeng Pan, Gregory A. Newby, Michael Wornow, Rachel Burt, Jonathan C. Chen, Jeffrey R. Holt†, David R. Liu1†  * co-first authors,  †co-senior authors

January 13, 2020

Efficient viral transduction in mouse inner ear hair cells with utricle injection and AAV9-PHP.B

July 03, 2019

Allele-specific gene editing prevents deafness in a model of dominant progressive hearing loss.

György B, Nist-Lund C, Pan B, Asai Y, Karavitaki KD, Kleinstiver BP, Garcia SP, Zaborowski MP, Solanes P, Spataro S, Schneider BL, Joung JK, Géléoc GSG, **Holt JR, Corey DP.   Nat Med. 2019 Jul;25(7):1123-1130.

**corresponding author

January 22, 2019

Improved TMC1 gene therapy restores hearing and balance in mice with genetic inner ear disorders

Nist-Lund CA, Pan B, Patterson A, Asai Y, Chen T, Zhou W, Zhu H, Romero S, Resnik J, Polley DB, Géléoc GS, Holt JR.

Nat Commun. 2019 Jan 22;10(1):236. doi: 10.1038/s41467-018-08264-w.

January 11, 2019

Split otoferlins reunited

Holt JR, Geleoc GS. EMBO Mol Med. 2019 Jan;11(1). pii: e9995. doi: 10.15252/emmm.201809995.

October 05, 2018

Function and Dysfunction of TMC Channels in Inner Ear Hair Cells

Corey DP, Akyuz N, Holt JR.  Cold Spring Harb Perspect Med. 2018 Oct 5. pii: a033506. doi: 10.1101/cshperspect.a033506.

August 22, 2018

TMC1 Forms the Pore of Mechanosensory Transduction Channels in Vertebrate Inner Ear Hair Cells

Bifeng Pan, Nurunisa Akyuz, Xiao-Ping Liu, Yukako Asai, Carl Nist-Lund, Kiyoto Kurima, Bruce H. Derfler, Bence Gyorgy, Walrati Limapichat, Sanket Walujkar, Lahiru N. Wimalasena, Marcos Sotomayor, David P. Corey, and Jeffrey R. Holt, Neuron, 2018 Aug 22;99(4):736-753

August 14, 2018

Transgenic Tmc2 expression preserves inner ear hair cells and vestibular function in mice lacking Tmc1

Asai Y, Pan B, Nist-Lund C, Galvin A, Lukashkin AN, Lukashkina VA, Chen T, Zhou W, Zhu H, Russell IJ, Holt JR, Géléoc GSG. Scientific Reports, 2018 Aug 14;8(1):12124.

August 14, 2018

Tmc2 expression partially restores auditory function in a mouse model of DFNB7/B11 deafness caused by loss of Tmc1 function

Nakanishi H, Kurima K, Pan B, Wangemann P, Fitzgerald TS, Géléoc GS, Holt JR, Griffith AJ.

Scientific Reports, 2018 Aug 14;8(1):12125.

July 18, 2018

Regenerating hair cells in vestibular sensory epithelia from humans

Taylor RR, Filia A, Paredes U, Asai Y, Holt JR, Lovett M, Forge A.

eLife, 2018 Jul 18;7. pii: e34817.

December 20, 2017

​Treatment of autosomal dominant hearing loss by in vivo delivery of genome editing agents

Gao X, Tao Y, Lamas V, Huang M, Yeh WH, Pan B, Hu YJ, Hu JH, Thompson DB, Shu Y, Li Y, Wang H, Yang S, Xu Q, Polley DB, Liberman MC, Kong WJ, Holt JR, Chen ZY, Liu DR.  Nature. 2017 Dec 20. 

August 16, 2017

Emerging Gene Therapies for Genetic Hearing Loss.

Ahmed, H., Shubina-Oleinik, O., & Holt, J. R. (2017). JARO

May 01, 2017

Generation of inner ear organoids containing functional hair cells from human pluripotent stem cells.

Koehler KR, Nie J, Longworth-Mills E, Liu XP, Lee J, Holt JR, Hashino E.

Nature Biotechnol. 2017 Jun;35(6):583-589. 

February 06, 2017

Gene therapy restores auditory and vestibular function in a mouse model of Usher syndrome type 1c.

Pan B, Askew C, Galvin A, Heman-Ackah S, Asai Y, Indzhykulian AA, Jodelka FM, Hastings ML, Lentz JJ, Vandenberghe LH, Holt JR, Géléoc GS.

Nature Biotech. 2017 Mar;35(3):264-272. 

February 06, 2017

A synthetic AAV vector enables safe and efficient gene transfer to the mammalian inner ear.

Landegger LD, Pan B, Askew C, Wassmer SJ, Gluck SD, Galvin A, Taylor R, Forge A, Stankovic KM, Holt JR, Vandenberghe LH. Nature Biotech. 2017 Mar;35(3):280-284. 

October 26, 2016

Are TMCs the Mechanotransduction Channels of Vertebrate Hair Cells?

Corey DP, Holt JR. J Neurosci. 2016 Oct 26;36(43):10921-10926.

May 26, 2016

RNA Interference Prevents Autosomal-Dominant Hearing Loss.  

S.B. Shibata, P.T. Ranum, H. Moteki, B. Pan, A.T. Goodwin, S.S. Goodman, P.J. Abbas, J.R. Holt, R.J. Smith.  Am J Hum Genet.  98(6):1101-13.  2016. 

May 24, 2016

Functional development of mechanosensitive hair cells in stem cell-derived organoids parallels native vestibular hair cells.  

X.P. Liu, K.R. Koehler, A.M. Mikosz, E. Hashino, J.R. Holt.  Nature Comms  7:11508. doi: 10.1038/ncomms11508.  2016 

Plug-N-Play: Mechanotransduction Goes Modular.

N. Akyuz, J.R. Holt. Neuron 89:1128-30. 2016. 

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 

Tmc gene therapy restores auditory function in deaf mice. 

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 and Holt JR. Science Trans Med. 2015 Jul 8;7(295) 

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

Kim YH, Holt JR.  J Gen Physiol. 142(3):207-23, 2013. 

Sound Strategies for Hearing Restoration.

Géléoc GSG and Holt JR. Science 344 no. 6184, 2014. 

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

Horwitz GC, Risner-Janiczek JR, Holt JR.  J Gen Physiol.  143(4):481-97, 2014. 

TMC function in hair cell transduction. 

Holt JR, Pan B, Koussa MA, Asai Y. Hear Res.  2014. 

May 01, 2014

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.  2013 

December 10, 2013

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 

September 01, 2013

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.

July 18, 2013

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, 79(3):504-15. 

October 01, 2012

Gene therapy for deaf mice goes viral.

Holt JR, Vandenberghe LH. Mol Ther. 2012 Oct;20(10):1836-7.

August 22, 2012

TMC1 and TMC2: key partners in hearing and vestibular function.

Géléoc GS. Med Sci (Paris). 2012, 28(8-9):701-3. 

July 11, 2012

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, 32(28):9485-98.

April 11, 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, 108(1):175-86.

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For a full list of Holt/Geleoc Lab publications, please click on this link: 
PubMed Holt/Geleoc Lab
 2018 by Holt/Géléoc Lab