Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation

Search Page

Filters

My Custom Filters

Edit custom filters

Results by year

Table representation of search results timeline featuring number of search results per year.

Year Number of Results
1994 1
1995 2
1997 2
1998 1
1999 6
2000 3
2002 3
2003 3
2004 2
2006 3
2007 4
2008 2
2009 5
2010 3
2011 4
2012 3
2013 3
2014 5
2015 4
2016 5
2017 5
2018 7
2019 8
2020 8
2021 7
2022 6
2023 6
2024 3
2025 8
2026 1

Publication date

Text availability

Article attribute

Article type

Additional filters

Article Language

Species

Sex

Age

Other

Search Results

113 results

Results by year

Filters applied: . Clear all
Page 1
AAV-mediated exon skipping therapy for Usher syndrome, type 2A.
Mauriac SA, Lee J, Zhang J, Jin J, Nist-Lund C, Martin CT, Von Muhlenbrock CR, O'Malley S, Chaves MG, Madison MA, Foster K, Long S, Jacobs C, Kumar SR, Ramirez S, Ichida J, Koehler KR, Holt JR, Géléoc GSG. Mauriac SA, et al. Among authors: holt jr, geleoc gsg. Mol Ther. 2026 Jan 7;34(1):179-202. doi: 10.1016/j.ymthe.2025.09.038. Epub 2025 Sep 23. Mol Ther. 2026. PMID: 40994011
There are four clinical subtypes, USH1-4, which are associated with mutations in genes important for structure, function, and survival of photoreceptor cells in the retina and sensory hair cells in the inner ear. Genetic mutations in the USH2A gene, which enc …
There are four clinical subtypes, USH1-4, which are associated with mutations in genes important for structure, function, and survival of ph …
A contemporary view of mechanosensory transduction in auditory hair cells.
Mun M, Holt JR. Mun M, et al. Among authors: holt jr. Biophys J. 2025 Dec 16;124(24):4407-4423. doi: 10.1016/j.bpj.2025.06.015. Epub 2025 Jun 16. Biophys J. 2025. PMID: 40528344 Review.
The ability to transduce the mechanical stimuli that initiate vertebrate hearing and balance is enabled by the stereociliary tips of inner ear hair cells, where a complex protein machine serves as a nexus for mechanosensory transduction (MT). The culmination of over …
The ability to transduce the mechanical stimuli that initiate vertebrate hearing and balance is enabled by the stereociliary tips of inner e …
Endocochlear potential contributes to hair cell death in TMPRSS3 hearing loss.
Shearer AE, Chen YS, Rouse SL, Wang X, Marin Fermin J, Booth KT, Moawad J, Libiran NB, Li J, Kim HY, Hoa M, Olszewski R, Lei JY, Cabrera E, Totten DJ, Zhao B, Holt JR, Nelson RF. Shearer AE, et al. Among authors: holt jr. J Clin Invest. 2025 Jul 17;135(18):e186395. doi: 10.1172/JCI186395. eCollection 2025 Sep 16. J Clin Invest. 2025. PMID: 40674144 Free PMC article.
Previous work has shown that Tmprss3Y260X/Y260X mice exhibit normal hair cell development, mechanosensory transduction, and spiral ganglion patterning, but experience rapid hair cell death from P12 to P14 at the onset of hearing. ...Furthermore, system …
Previous work has shown that Tmprss3Y260X/Y260X mice exhibit normal hair cell development, mechanosensory transduction, and sp …
AAV gene therapy rescues hearing and balance in a model of CLIC5 deafness.
Hahn R, Taiber S, Shubina-Oleinik O, Géléoc GSG, Holt JR, Avraham KB. Hahn R, et al. Among authors: holt jr, geleoc gsg. EMBO Mol Med. 2025 Sep;17(9):2233-2257. doi: 10.1038/s44321-025-00275-7. Epub 2025 Aug 26. EMBO Mol Med. 2025. PMID: 40859056 Free PMC article.
We report a robust restoration of CLIC5 expression using either construct, including prevention of morphological degeneration and preserving auditory and vestibular function. Interestingly, the self-complementary construct achieved comparable functional recovery to single- …
We report a robust restoration of CLIC5 expression using either construct, including prevention of morphological degeneration and preserving …
Mammalian TMC1 or 2 are necessary for scramblase activity in auditory hair cells.
Peineau T, Marcovich I, Rodriguez CVM, O'Malley S, Cui R, Ballesteros A, Holt JR. Peineau T, et al. Among authors: holt jr. Hear Res. 2025 May;460:109229. doi: 10.1016/j.heares.2025.109229. Epub 2025 Mar 6. Hear Res. 2025. PMID: 40073458
Sensory transduction in auditory hair cells gates mechanosensitive ion channels, converting sound information into electrical signals (Zheng and Holt, 2021). ...Here, we investigated lipid scramblase activity in live auditory hair cells w …
Sensory transduction in auditory hair cells gates mechanosensitive ion channels, converting sound information into elec …
Complexes of vertebrate TMC1/2 and CIB2/3 proteins form hair-cell mechanotransduction cation channels.
Giese APJ, Weng WH, Kindt KS, Chang HHV, Montgomery JS, Ratzan EM, Beirl AJ, Aponte Rivera R, Lotthammer JM, Walujkar S, Foster MP, Zobeiri OA, Holt JR, Riazuddin S, Cullen KE, Sotomayor M, Ahmed ZM. Giese APJ, et al. Among authors: holt jr. Elife. 2025 Jan 8;12:RP89719. doi: 10.7554/eLife.89719. Elife. 2025. PMID: 39773557 Free PMC article.
Overall, our work demonstrates that intact CIB2/3 and TMC1/2 complexes are integral to hair-cell MET function in vertebrate mechanosensory epithelia....
Overall, our work demonstrates that intact CIB2/3 and TMC1/2 complexes are integral to hair-cell MET function in vertebrate me …
Complexes of vertebrate TMC1/2 and CIB2/3 proteins form hair-cell mechanotransduction cation channels.
Giese APJ, Weng WH, Kindt KS, Chang HHV, Montgomery JS, Ratzan EM, Beirl AJ, Rivera RA, Lotthammer JM, Walujkar S, Foster MP, Zobeiri OA, Holt JR, Riazuddin S, Cullen KE, Sotomayor M, Ahmed ZM. Giese APJ, et al. Among authors: holt jr. bioRxiv [Preprint]. 2024 Jul 5:2023.05.26.542533. doi: 10.1101/2023.05.26.542533. bioRxiv. 2024. Update in: Elife. 2025 Jan 08;12:RP89719. doi: 10.7554/eLife.89719. PMID: 37398045 Free PMC article. Updated. Preprint.
Overall, our work demonstrates that intact CIB2/3 and TMC1/2 complexes are integral to hair-cell MET function in vertebrate mechanosensory epithelia....
Overall, our work demonstrates that intact CIB2/3 and TMC1/2 complexes are integral to hair-cell MET function in vertebrate me …
Sensory transduction in auditory hair cells-PIEZOs can't touch this.
Holt JR, Fettiplace R, Müller U. Holt JR, et al. J Gen Physiol. 2024 Jun 3;156(6):e202413585. doi: 10.1085/jgp.202413585. Epub 2024 May 10. J Gen Physiol. 2024. PMID: 38727631 Free PMC article.
In this Viewpoint, Holt, Fettiplace, and Muller weigh the evidence supporting a role for PIEZO and TMC channels in mechanosensory transduction in inner ear hair cells....
In this Viewpoint, Holt, Fettiplace, and Muller weigh the evidence supporting a role for PIEZO and TMC channels in mechanosensory transducti …
113 results