HOLT Jeffrey R.

Project status: closed

Awarded the Scientific Grand Prize in 2019, Professor Jeffrey R. Holt, Professor of Otolaryngology and Neurology at Boston Children’s Hospital and Harvard Medical School in the USA, has discovered the role of a key protein in the hearing system: TMC1.

It allows sound waves perceived by the ear to be converted into electrical signals which the brain can then decode and process.

Jeffrey R. Holt also puts huge effort into trials of gene therapies aimed at restoring auditory and vestibular function to persons with hearing loss.

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Professeur Jeffrey R. Holt
Investigator
Boston Children’s Hospital, Boston, MS, USA

Related scientific publication(s) :

• 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 Liu. In vivo base editing restores sensory transduction and transiently improves auditory function in a mouse model of recessive deafness. Sci Transl Med. 2020 Jun 3;12(546):eaay9101. doi: 10.1126/scitranslmed.aay9101.  
• John Lee, Carl Nist-Lund, Paola Solanes, Hannah Goldberg, Jason Wu, Bifeng Pan, Bernard L Schneider, Jeffrey R Holt. Efficient viral transduction in mouse inner ear hair cells with utricle injection and AAV9-PHP.B.Hear Res. 2020 Sep 1;394:107882. doi: 10.1016/j.heares.2020.107882. Epub 2020 Jan 13. 
• Shahar Taiber, Roie Cohen, Ofer Yizhar-Barnea, David Sprinzak, Jeffrey R Holt, Karen B Avraham. Neonatal AAV gene therapy rescues hearing in a mouse model of SYNE4 deafness. EMBO Mol Med 2021 Feb 5;13(2):e13259.doi: 10.15252/emmm.202013259. Epub 2020 Dec 22.
• Jason Wu, Paola Solanes, Carl Nist-Lund, Sofia Spataro, Olga Shubina-Oleinik , Irina Marcovich, Hannah Goldberg, Bernard L Schneider, Jeffrey R Holt. Single and dual vector gene therapy with AAV9-PHP.B rescues hearing in Tmc1 mutant mice. Mol Ther. 2021 Mar 3;29(3):973-988. doi: 10.1016/j.ymthe.2020.11.016. Epub 2020 Nov 17. 
• Wang Zheng, Jeffrey R Holt. The Mechanosensory Transduction Machinery in Inner Ear Hair Cells. Annu Rev Biophys. 2021 May 6;50:31-51. doi: 10.1146/annurev-biophys-062420-081842. Epub 2020 Dec 7. 
• John Lee, Kosuke Kawai, Jeffrey R Holt, Gwenaelle Geleoc. Sensory transduction is required for normal development and maturation ofcochlear inner hair cell synapses. Elife. 2021 Nov 4;10:e69433. doi: 10.7554/eLife.69433. 
• Olga Shubina-Oleinik, Carl Nist-Lund, Courtney French, Shira Rockowitz, A Eliot Shearer, Jeffrey R Holt. Dual-vector gene therapy restores cochlear amplification and auditory sensitivity in a mouse model of DFNB16 hearing loss. Sci Adv. 2021 Dec 17;7(51):eabi7629. doi: 10.1126/sciadv.abi7629. Epub 2021 Dec 15. 
• Jeffrey R Holt, Mélanie Tobin, Johannes Elferich, Eric Gouaux, Angela Ballesteros, Zhiqiang Yan, Zubair M Ahmed, Teresa Nicolson. Putting the Pieces Together: the Hair Cell Transduction Complex. J Assoc Res Otolaryngol. 2021 Dec;22(6):601-608. doi: 10.1007/s10162-021-00808-0. Epub 2021 Oct 6. 
• Irina Marcovich, Nicholas K Baer , Olga Shubina-Oleinik, Rachel Eclov, Clayton W Beard, Jeffrey R Holt. Optimized AAV Vectors for TMC1 Gene Therapy in a Humanized Mouse Model of DFNB7/11. Biomolecules. 2022 Jun 29;12(7):914. doi: 10.3390/biom12070914.