ArticleMolecular and cellular biology2005
Deafness and cochlear fibrocyte alterations in mice deficient for the inner ear protein otospiralin.
Article in Molecular and cellular biology, 2005. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
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Who cites it
31 citing papers in PubMed, 66 citations in OpenAlex.
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- Translational and interdisciplinary insights into presbyacusis: A multidimensional disease.Hearing research · 2021Review
- Immunologic Profiling of the Atlantic Salmon Gill by Single Nuclei Transcriptomics.Frontiers in immunology · 2021Article
- Gene duplication and functional divergence of the zebrafish otospiralin genes.Development genes and evolution · 2020Article
- Role of Dach1 revealed using a novel inner ear-specific Dach1-knockdown mouse model.Biology open · 2019Article
- Clinical and Genome-wide Analysis of Cisplatin-induced Tinnitus Implicates Novel Ototoxic Mechanisms.Clinical cancer research : an official journal of the American Association for Cancer Research · 2019Article
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- Stereocilia morphogenesis and maintenance through regulation of actin stability.Seminars in cell & developmental biology · 2017Review
- Regulation of Fn14 Receptor and NF-κB Underlies Inflammation in Meniere's Disease.Frontiers in immunology · 2017Article
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- Noise trauma and systemic application of the selective glucocorticoid receptor modulator compound A.Journal of negative results in biomedicine · 2016Article
- Phylogenetic analysis of otospiralin protein.Advanced biomedical research · 2016Article
- Deficiency of transcription factor Brn4 disrupts cochlear gap junction plaques in a model of DFN3 non-syndromic deafness.PloS one · 2014Article
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- Spontaneous recovery of cochlear fibrocytes after severe degeneration caused by acute energy failure.Frontiers in pharmacology · 2014Review
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- Current status and prospects of gene therapy for the inner ear.Human gene therapy · 2011Review
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Authors and funding
9 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
In the cochlea, the mammalian auditory organ, fibrocytes of the mesenchymal nonsensory regions play important roles in cochlear physiology, including the maintenance of ionic and hydric components in the endolymph. Occurrence of human deafness in fibrocyte alterations underlines their critical roles in auditory function. We recently described a novel gene, Otos, which encodes otospiralin, a small protein of unknown function that is produced by the fibrocytes of the cochlea and vestibule. We now have generated mice with deletion of Otos and found that they show moderate deafness, with no frequency predominance. Histopathology revealed a degeneration of type II and IV fibrocytes, while hair cells and stria vascularis appeared normal. Together, these findings suggest that impairment of fibrocytes caused by the loss in otospiralin leads to abnormal cochlear physiology and auditory function. This moderate dysfunction may predispose to age-related hearing loss.
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