ArticleScientific reports2018
Transgenic Tmc2 expression preserves inner ear hair cells and vestibular function in mice lacking Tmc1.
Article in Scientific reports, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 19 papers.
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Who cites it
19 citing papers in PubMed, 36 citations in OpenAlex.
- Evolutionary tuning of an auditory transduction channel.Current biology : CB · 2026Article
- Distinct adaptation and ancestral retention signals in African and European indigenous cattle genomes.Communications biology · 2026Article
- Functional contributions of quantal and non-quantal hair cell synaptic transmission in the vestibular periphery.bioRxiv : the preprint server for biology · 2025Article
- Pharmacological regeneration of sensory hair cells restores afferent innervation and vestibular function.The Journal of clinical investigation · 2024Article
- LOXHD1 is indispensable for maintaining TMC1 auditory mechanosensitive channels at the site of force transmission.Nature communications · 2024Article
- LOXHD1 is indispensable for coupling auditory mechanosensitive channels to the site of force transmission.Research square · 2024Article
- Characterization of 3,3'-iminodipropionitrile (IDPN) damaged utricle transcriptome in the adult mouse utricle.Frontiers in molecular neuroscience · 2024Article
- Novel autosomal dominant TMC1 variants linked to hearing loss: insight into protein-lipid interactions.BMC medical genomics · 2023Article
- Loss of Pex1 in Inner Ear Hair Cells Contributes to Cochlear Synaptopathy and Hearing Loss.Cells · 2022Article
- Regulation of membrane homeostasis by TMC1 mechanoelectrical transduction channels is essential for hearing.Science advances · 2022Article
- Mechanisms in cochlear hair cell mechano-electrical transduction for acquisition of sound frequency and intensity.Cellular and molecular life sciences : CMLS · 2021Review
- The Mechanosensory Transduction Machinery in Inner Ear Hair Cells.Annual review of biophysics · 2021Review
- Expression of a membrane-targeted fluorescent reporter disrupts auditory hair cell mechanoelectrical transduction and causes profound deafness.Hearing research · 2021Article
- Article
- Dimensions of a Living Cochlear Hair Bundle.Frontiers in cell and developmental biology · 2021Article
- Direct Delivery of Antisense Oligonucleotides to the Middle and Inner Ear Improves Hearing and Balance in Usher Mice.Molecular therapy : the journal of the American Society of Gene Therapy · 2020Article
- Disruption ofThe Journal of neuroscience : the official journal of the Society for Neuroscience · 2020Article
- Function and Dysfunction of TMC Channels in Inner Ear Hair Cells.Cold Spring Harbor perspectives in medicine · 2019Review
- Tmc2 expression partially restores auditory function in a mouse model of DFNB7/B11 deafness caused by loss of Tmc1 function.Scientific reports · 2018Article
Corrections and comments
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Authors and funding
12 authors at 3 institutions in 2 countries.
Funding
Abstract
Recent work has demonstrated that transmembrane channel-like 1 protein (TMC1) is an essential component of the sensory transduction complex in hair cells of the inner ear. A closely related homolog, TMC2, is expressed transiently in the neonatal mouse cochlea and can enable sensory transduction in Tmc1-null mice during the first postnatal week. Both TMC1 and TMC2 are expressed at adult stages in mouse vestibular hair cells. The extent to which TMC1 and TMC2 can substitute for each other is unknown. Several biophysical differences between TMC1 and TMC2 suggest these proteins perform similar but not identical functions. To investigate these differences, and whether TMC2 can substitute for TMC1 in mature hair cells, we generated a knock-in mouse model allowing Cre-inducible expression of Tmc2. We assayed for changes in hair cell sensory transduction and auditory and vestibular function in Tmc2 knockin mice (Tm[Tmc2]) in the presence or absence of endogenous Tmc1, Tmc2 or both. Our results show that expression of Tm[TMC2] restores sensory transduction in vestibular hair cells and transiently in cochlear hair cells in the absence of TMC1. The cellular rescue leads to recovery of balance but not auditory function. We conclude that TMC1 provides some additional necessary function, not provided by TMC2.
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