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Jeffrey R. Holt

Boston Children's Hospital

51H-index
151Paper Count
1.0WCitation Count
Published Papers 48
Publication Date
Assessment of auditory and vestibular function and gene therapy in the Snell’s waltzer mouse model of human deafness and balance dysfunction
err2026-08-28
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errOAAI
errEyal Marton; Roni Hahn; Lior Bikovski; Gwenaëlle S. G. Géléoc; Jeffrey R. Holt; Matti Mintz; Karen B. Avraham
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AAV-Mediated Exon Skipping Therapy for Usher Syndrome, Type 2A.
err2025-09-23
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PREAI
errStephanie A. Mauriac; Jiyoon Lee; Jingyuan Zhang; Jiahe Jin; Carl Nist-Lund; Camille T. Martin; Cristobal R. Von Muhlenbrock; Sydney O’Malley; Mary G. Chaves; Margot A. Madison; Kate Foster; Shinong Long; Chris Jacobs; Sripriya Ravindra Kumar; Silvia Ramirez; Justin Ichida; Karl R. Koehler; Jeffrey R. Holt; Gwenaëlle S.G. Géléoc
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Endocochlear potential contributes to hair cell death in TMPRSS3 hearing loss
err2025-09-16
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errOAAI
errShearer, A. Eliot; Chen, Yuan-Siao; Rouse, Stephanie L.; Wang, Xiaohan; Fermin, Janmaris Marin; Booth, Kevin T. A.; Moawad, Jasmine; Libiran, Nicole Bianca; Li, Jinan; Kim, Hae-Young; Hoa, Michael; Olszewski, Rafal; Lei, Jing-Yu; Cabrera, Ernesto; Totten, Douglas J.; Zhao, Bo; Holt, Jeffrey R.; Nelson, Rick F.
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Structural and functional basis of mechanosensitive TMEM63 channelopathies
errNeuron
IF15
err2025-06-05
err0
PREAI
errWang Zheng; Augustus J. Lowry; Harper E. Smith; Jiale Xie; Shaun Rawson; Chen Wang; Jin Ou; Marcos Sotomayor; Tian-Min Fu; Huanghe Yang; Jeffrey R. Holt
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Mammalian TMC1 or 2 are necessary for scramblase activity in auditory hair cells
err2025-05-01
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PREAI
errPeineau, Thibault; Marcovich, Irina; Rodriguez, Cristobal von Muhlenbrock; O'Malley, Sydney; Cui, Runjia; Ballesteros, Angela; Holt, Jeffrey R.
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Complexes of vertebrate TMC1/2 and CIB2/3 proteins form hair-cell mechanotransduction cation channels
err2025-01-08
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errOAAI
errGiese, Arnaud P. J.; Weng, Wei-Hsiang; Kindt, Katie S.; Chang, Hui Ho Vanessa; Montgomery, Jonathan S.; Ratzan, Evan M.; Beirl, Alisha J.; Aponte Rivera, Roberto; Lotthammer, Jeffrey M.; Walujkar, Sanket; Foster, Mark P.; Zobeiri, Omid A.; Holt, Jeffrey R.; Riazuddin, Saima; Cullen, Kathleen E.; Sotomayor, Marcos; Ahmed, Zubair M.
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Sensory transduction in auditory hair cells-PIEZOs can't touch this
err2024-05-10
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errOAAI
errHolt, Jeffrey R.; Fettiplace, Robert; Muller, Ulrich
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TMEM63 proteins function as monomeric high-threshold mechanosensitive ion channels
errNEURON
IF15
err2023-10-01
err32
PREAI
errZheng, Wang; Rawson, Shaun; Shen, Zhangfei; Tamilselvan, Elakkiya; Smith, Harper E.; Halford, Julia; Shen, Chen; Murthy, Swetha E.; Ulbrich, Maximilian H.; Sotomayor, Marcos; Fu, Tian-Min; Holt, Jeffrey R.
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Delivery of gene therapy through a cerebrospinal fluid conduit to rescue hearing in adult mice
err2023-06-28
err15
PREAI
errMathiesen, Barbara K.; Miyakoshi, Leo M.; Cederroth, Christopher R.; Tserga, Evangelia; Versteegh, Corstiaen; Bork, Peter A. R.; Hauglund, Natalie L.; Gomolka, Ryszard Stefan; Mori, Yuki; Edvall, Niklas K.; Rouse, Stephanie; Mollgard, Kjeld; Holt, Jeffrey R.; Nedergaard, Maiken; Canlon, Barbara
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pH regulates potassium conductance and drives a constitutive proton current in human TMEM175
err2022-03-25
err27
errOAAI
errZheng, Wang; Shen, Chen; Wang, Longfei; Rawson, Shaun; Xie, Wen Jun; Nist-Lund, Carl; Wu, Jason; Shen, Zhangfei; Xia, Shiyu; Holt, Jeffrey R.; Wu, Hao; Fu, Tian-Min
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Dual-vector gene therapy restores cochlear amplification and auditory sensitivity in a mouse model of DFNB16 hearing loss
err2021-12-17
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errOAAI
errShubina-Oleinik, Olga; Nist-Lund, Carl; French, Courtney; Rockowitz, Shira; Shearer, A. Eliot; Holt, Jeffrey R.
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Sensory transduction is required for normal development and maturation of cochlear inner hair cell synapses
err2021-11-04
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PREAI
errLee, John; Kawai, Kosuke; Holt, Jeffrey R.; Geleoc, Gwenaelle Sg
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Single and Dual Vector Gene Therapy with AAV9-PHP.B Rescues Hearing in Tmc1 Mutant Mice
err2021-03-01
err42
errOAAI
errWu, Jason; Solanes, Paola; Nist-Lund, Carl; Spataro, Sofia; Shubina-Oleinik, Olga; Marcovich, Irina; Goldberg, Hannah; Schneider, Bernard L.; Holt, Jeffrey R.
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Neonatal AAV gene therapy rescues hearing in a mouse model of SYNE4 deafness
err2020-12-22
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errOAAI
errTaiber, Shahar; Cohen, Roie; Yizhar-Barnea, Ofer; Sprinzak, David; Holt, Jeffrey R.; Avraham, Karen B.
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In vivo base editing restores sensory transduction and transiently improves auditory function in a mouse model of recessive deafness
err2020-06-03
err128
errOAAI
errYeh, Wei-Hsi; Shubina-Oleinik, Olga; Levy, Jonathan M.; Pan, Bifeng; Newby, Gregory A.; Wornow, Michael; Burt, Rachel; Chen, Jonathan C.; Holt, Jeffrey R.; Liu, David R.
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Continuous evolution of base editors with expanded target compatibility and improved activity (vol 37, pg 1070, 2019)
err2019-08-12
err7
errOAAI
errThuronyi, Benjamin W.; Koblan, Luke W.; Levy, Jonathan M.; Yeh, Wei-Hsi; Zheng, Christine; Newby, Gregory A.; Wilson, Christopher; Bhaumik, Mantu; Shubina-Oleinik, Olga; Holt, Jeffrey R.; Liu, David R.
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Continuous evolution of base editors with expanded target compatibility and improved activity
err2019-07-22
err269
errOAAI
errThuronyi, Benjamin W.; Koblan, Luke W.; Levy, Jonathan M.; Yeh, Wei-Hsi; Zheng, Christine; Newby, Gregory A.; Wilson, Christopher; Bhaumik, Mantu; Shubina-Oleinik, Olga; Holt, Jeffrey R.; Liu, David R.
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Allele-specific gene editing prevents deafness in a model of dominant progressive hearing loss
err2019-07-03
err154
errOAAI
errGyorgy, Bence; Nist-Lund, Carl; Pan, Bifeng; Asai, Yukako; Karavitaki, K. Domenica; Kleinstiver, Benjamin P.; Garcia, Sara P.; Zaborowski, Mikolaj P.; Solanes, Paola; Spataro, Sofia; Schneider, Bernard L.; Joung, J. Keith; Geleoc, Gwenaelle S. G.; Holt, Jeffrey R.; Corey, David P.
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Improved TMC1 gene therapy restores hearing and balance in mice with genetic inner ear disorders (vol 10, 236, 2019)
err2019-02-08
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errOAAI
errNist-Lund, Carl A.; Pan, Bifeng; Patterson, Amy; Asai, Yukako; Chen, Tianwen; Zhou, Wu; Zhu, Hong; Romero, Sandra; Resnik, Jennifer; Polley, Daniel B.; Geleoc, Gwenaelle S.; Holt, Jeffrey R.
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