Cited by 114Hearing, Cochlea, Tinnitus, GeneticsCongenital Ear and Nasal AnomaliesHearing Loss and Rehabilitation

miR-96 regulates the progression of differentiation in mammalian cochlear inner and outer hair cells

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Kuhn, Stephanie, Johnson, Stuart L., Furness, David N., Chen, Jing, Ingham, Neil, Hilton, Jennifer M., Steffes, Georg, Lewis, Morag A. +6 more·2011·Proceedings of the National Academy of Sciences

Abstract

MicroRNAs (miRNAs) are small noncoding RNAs able to regulate a broad range of protein-coding genes involved in many biological processes. miR-96 is a sensory organ-specific miRNA expressed in the mammalian cochlea during development. Mutations in miR-96 cause nonsyndromic progressive hearing loss in humans and mice. The mouse mutant diminuendo has a single base change in the seed region of the Mir96 gene leading to widespread changes in the expression of many genes. We have used this mutant to explore the role of miR-96 in the maturation of the auditory organ. We found that the physiological development of mutant sensory hair cells is arrested at around the day of birth, before their biophysical differentiation into inner and outer hair cells. Moreover, maturation of the hair cell stereocilia bundle and remodelling of auditory nerve connections within the cochlea fail to occur in miR-96 mutants. We conclude that miR-96 regulates the progression of the physiological and morphological differentiation of cochlear hair cells and, as such, coordinates one of the most distinctive functional refinements of the mammalian auditory system.

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Crossref · 10.1073/pnas.1016646108

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Authors
Kuhn, S., Johnson, S. L., Furness, D. N., Chen, J., Ingham, N., Hilton, J. M., Steffes, G., Lewis, M. A., Zampini, V., Hackney, C. M., Masetto, S., Holley, M. C., Steel, K. P., & Marcotti, W.
Affiliations
  • University of Sheffield (GB)
  • Keele University (GB)
  • Wellcome Sanger Institute (GB)
  • University of Pavia (IT)
Venue
Proceedings of the National Academy of Sciences
Published
2011-01-18
Volume
108
Issue
6
Pages
2355-2360
ISSN
  • 0027-8424 (ISSN-L)
  • 1091-6490
OpenAlex
W2027444951
Cited by
114
References
51
Citations by year
2026: 3 · 2025: 8 · 2024: 10 · 2023: 3 · 2022: 4 · 2021: 3 · 2020: 7 · 2019: 4 · 2018: 8 · 2017: 9 · 2016: 7 · 2015: 6 · 2014: 6 · 2013: 14 · 2012: 15
FWCI
7.24
Type
journal article
Language
en
Open access
Locations
  • Proceedings of the National Academy of Sciences (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
  • Research Portal (King's College London) (submittedVersion)
Funders
  • Wellcome Trust (077189, 088719)
  • Deafness Research UK
  • Henry Smith Charity
  • University of Sheffield
  • Medical Research Council (MC_qA137918)
Topics
  • Life Sciences › Neuroscience › Sensory Systems › Hearing, Cochlea, Tinnitus, Genetics
  • Health Sciences › Medicine › Genetics › Congenital Ear and Nasal Anomalies
  • Life Sciences › Neuroscience › Cognitive Neuroscience › Hearing Loss and Rehabilitation
Keywords
miR-96, microRNA, cochlear hair cells, inner hair cells, outer hair cells, hair cell differentiation, progressive hearing loss
MeSH terms
Animals, Cell Differentiation, Hair Cells, Auditory, Inner, Mice, Mutant Strains, Mutation, Organ Specificity, Hair Cells, Auditory, Outer, MicroRNAs, Mice

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