ArticleNature2008
Functional auditory hair cells produced in the mammalian cochlea by in utero gene transfer.
Article in Nature, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 164 papers.
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Who cites it
164 citing papers in PubMed, 270 citations in OpenAlex.
- Assessment of auditory and vestibular function and gene therapy in the Snell's waltzer mouse model of human deafness and balance dysfunction.Mammalian genome : official journal of the International Mammalian Genome Society · 2026Article
- Mapping the global trends and hotspots of research on gene therapy and hair cell regeneration for hearing loss: a comprehensive data-mining-based study.Gene therapy · 2026Article
- Galectin and Myc enable cochlear progenitor expansion in vitro and in vivo.bioRxiv : the preprint server for biology · 2026Article
- Dominant and recessive ATOH1 variants cause distinct neurodevelopmental disorders with hearing loss.American journal of human genetics · 2026Article
- An engineered ferritin nanocage co-delivery system for targeted hair cells protection and hearing loss attenuation.Theranostics · 2026Article
- Implication of GPRASP2 in the Proliferation and Hair Cell-Forming of Cochlear Supporting Cells.Cell proliferation · 2025Article
- Exploring AAV-Mediated Gene Therapy for Inner Ear Diseases: from Preclinical Success to Clinical Potential.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2025Review
- Wnt/PKC Signaling Inhibits Sensory Hair Cell Formation in the Developing Mammalian Cochlea.Cells · 2025Article
- Conditional Overexpression of Net1 Enhances the Trans-Differentiation of Lgr5Cell proliferation · 2025Article
- Genetic and pharmacologic alterations of claudin9 levels suffice to induce functional and mature inner hair cells.bioRxiv : the preprint server for biology · 2024Article
- Precise genetic control of ATOH1 enhances maturation of regenerated hair cells in the mature mouse utricle.Nature communications · 2024Article
- AAV-mediated Gene Cocktails Enhance Supporting Cell Reprogramming and Hair Cell Regeneration.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2024Article
- Stem Cell-Based Hair Cell Regeneration and Therapy in the Inner Ear.Neuroscience bulletin · 2024Review
- Exploring delivery systems for targeted nanotechnology-based gene therapy in the inner ear.Therapeutic delivery · 2024Review
- Article
- The role of Espin in the stereocilia regeneration and protection in Atoh1-overexpressed cochlear epithelium.Cell proliferation · 2023Article
- Article
- Sufu- and Spop-mediated regulation of Gli2 is essential for the control of mammalian cochlear hair cell differentiation.Proceedings of the National Academy of Sciences of the United States of America · 2022Article
- The Bioactive Phenolic Agents Diaryl Ether CVB2-61 and Diarylheptanoid CVB4-57 as Connexin Hemichannel Blockers.Pharmaceuticals (Basel, Switzerland) · 2022Article
- Three distinctProceedings of the National Academy of Sciences of the United States of America · 2022Article
104 more citing papers are in PubMed but not listed here.
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Authors and funding
5 authors at 4 institutions in 1 country.
Funding
Abstract
Sensory hair cells in the mammalian cochlea convert mechanical stimuli into electrical impulses that subserve audition. Loss of hair cells and their innervating neurons is the most frequent cause of hearing impairment. Atonal homologue 1 (encoded by Atoh1, also known as Math1) is a basic helix-loop-helix transcription factor required for hair-cell development, and its misexpression in vitro and in vivo generates hair-cell-like cells. Atoh1-based gene therapy to ameliorate auditory and vestibular dysfunction has been proposed. However, the biophysical properties of putative hair cells induced by Atoh1 misexpression have not been characterized. Here we show that in utero gene transfer of Atoh1 produces functional supernumerary hair cells in the mouse cochlea. The induced hair cells display stereociliary bundles, attract neuronal processes and express the ribbon synapse marker carboxy-terminal binding protein 2 (refs 12,13). Moreover, the hair cells are capable of mechanoelectrical transduction and show basolateral conductances with age-appropriate specializations. Our results demonstrate that manipulation of cell fate by transcription factor misexpression produces functional sensory cells in the postnatal mammalian cochlea. We expect that our in utero gene transfer paradigm will enable the design and validation of gene therapies to ameliorate hearing loss in mouse models of human deafness.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.