ArticleThe Journal of cell biology2004
Radixin deficiency causes deafness associated with progressive degeneration of cochlear stereocilia.
Article in The Journal of cell biology, 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 79 papers.
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Who cites it
79 citing papers in PubMed, 145 citations in OpenAlex.
- LOXHD1b knockout alters swimming behavior in zebrafish.Cell and tissue research · 2025Article
- Histamine H3 Receptor Antagonists Influence the Directional Growth of Type II Spiral Ganglion Neurites Within the Developing Cochlea of C57BL/6 Mice.Neurochemical research · 2025Article
- MSRB3 antioxidant activity is necessary for inner ear cuticular plate structure and hair bundle integrity.Disease models & mechanisms · 2025Article
- OSBPL2 deficiency inhibits Rho/ROCK2/p-ERM signaling and impairs actin cytoskeletal regulation in auditory cells.Journal of biomedical research · 2025Article
- Dominant effect of a single amino acid mutation in the motor domain of myosin VI on hearing in mice.Experimental animals · 2025Article
- Whole-genome sequencing, as a powerful diagnostic tool in hearing loss, reveals novel variants in PTPRQ missed by whole-exome sequencing.BMC medical genomics · 2025Article
- Radixin: Roles in the Nervous System and Beyond.Biomedicines · 2024Review
- Keratan sulfate, an electrosensory neurosentient bioresponsive cell instructive glycosaminoglycan.Glycobiology · 2024Review
- Critical role of TPRN rings in the stereocilia for hearing.Molecular therapy : the journal of the American Society of Gene Therapy · 2024Article
- Inherited human ezrin deficiency impairs adaptive immunity.The Journal of allergy and clinical immunology · 2023Article
- Article
- The morphological and functional diversity of apical microvilli.Journal of anatomy · 2023Review
- Article
- Human deafness-associated variants alter the dynamics of key molecules in hair cell stereocilia F-actin cores.Human genetics · 2022Review
- CaMolecular biology of the cell · 2022Article
- MANF supports the inner hair cell synapse and the outer hair cell stereocilia bundle in the cochlea.Life science alliance · 2022Article
- Monoallelic loss of the F-actin-binding protein radixin facilitates startle reactivity and pre-pulse inhibition in mice.Frontiers in cell and developmental biology · 2022Article
- Radixin modulates the function of outer hair cell stereocilia.Communications biology · 2020Article
- Prominin-1-Radixin axis controls hepatic gluconeogenesis by regulating PKA activity.EMBO reports · 2020Article
- A cryo-tomography-based volumetric model of the actin core of mouse vestibular hair cell stereocilia lacking plastin 1.Journal of structural biology · 2020Article
19 more citing papers are in PubMed but not listed here.
Corrections and comments
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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
Ezrin/radixin/moesin (ERM) proteins cross-link actin filaments to plasma membranes to integrate the function of cortical layers, especially microvilli. We found that in cochlear and vestibular sensory hair cells of adult wild-type mice, radixin was specifically enriched in stereocilia, specially developed giant microvilli, and that radixin-deficient (Rdx(-)(/)(-)) adult mice exhibited deafness but no obvious vestibular dysfunction. Before the age of hearing onset ( approximately 2 wk), in the cochlea and vestibule of Rdx(-)(/)(-) mice, stereocilia developed normally in which ezrin was concentrated. As these Rdx(-)(/)(-) mice grew, ezrin-based cochlear stereocilia progressively degenerated, causing deafness, whereas ezrin-based vestibular stereocilia were maintained normally in adult Rdx(-)(/)(-) mice. Thus, we concluded that radixin is indispensable for the hearing ability in mice through the maintenance of cochlear stereocilia, once developed. In Rdx(-)(/)(-) mice, ezrin appeared to compensate for radixin deficiency in terms of the development of cochlear stereocilia and the development/maintenance of vestibular stereocilia. These findings indicated the existence of complicate functional redundancy in situ among ERM proteins.
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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.