Publications
For a full list of Holt/Geleoc Lab publications, please click on this link:
August, 2023
TMEM63 proteins function as monomeric high-threshold mechanosensitive ion channels
Wang Zheng, Shaun Rawson, Zhangfei Shen, Elakkiya Tamilselvan, Harper E. Smith, Julia Halford, Chen Shen, Swetha E. Murthy, Maximilian H. Ulbrich, Marcos Sotomayor, Tian-Min Fu and Jeffrey R. Holt
May, 2023
Complexes of vertebrate TMC1/2 and CIB2/3 proteins form hair-cell mechanotransduction cation channels
Arnaud P J Giese, Wei-Hsiang Weng, Katie S Kindt, Hui Ho Vanessa Chang, Jonathan S Montgomery, Evan M Ratzan, Alisha J Beirl, Roberto Aponte Rivera, Jeffrey M Lotthammer, Sanket Walujkar, Mark P Foster, Omid A Zobeiri, Jeffrey R Holt, Saima Riazuddin, Kathleen E Cullen, Marcos Sotomayor, Zubair M Ahmed
June, 2023
Delivery of gene therapy through a cerebrospinal fluid conduit to rescue hearing in adult mice
Mathiesen BK, Miyakoshi LM, Cederroth CR, Tserga E, Versteegh C, Bork PAR, Hauglund NL, Gomolka RS, Mori Y, Edvall NK, Rouse S, Møllgård K, Holt JR, Nedergaard M, Canlon B.
April, 2023
Molecular earplugs to protect the inner ear
Mary Grace Chaves and Jeffrey R. Holt
December, 2022
Loss of Pex1 in Inner Ear Hair Cells Contributes to Cochlear Synaptopathy and Hearing Loss
Stephanie A. Mauriac, Thibault Peineau, Aamir Zuberi, Cathleen Lutz and Gwénaëlle S. G. Géléoc
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
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
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
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
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
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
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
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
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.
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
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
Plug-N-Play: Mechanotransduction Goes Modular.
N. Akyuz, J.R. Holt. Neuron 89:1128-30. 2016.
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.