ArticleJournal of the Association for Research in Otolaryngology : JARO2005
Characterization of vestibular dysfunction in the mouse model for Usher syndrome 1F.
Article in Journal of the Association for Research in Otolaryngology : JARO, 2005. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 23 papers.
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Who cites it
23 citing papers in PubMed, 36 citations in OpenAlex.
- An open-source tool for automated human-level circling behavior detection.Scientific reports · 2024Article
- Article
- Rescue of hearing by adenine base editing in a humanized mouse model of Usher syndrome type 1F.Molecular therapy : the journal of the American Society of Gene Therapy · 2023Article
- An Open-Source Tool for Automated Human-Level Circling Behavior Detection.bioRxiv : the preprint server for biology · 2023Article
- A variant in LMX1A causes autosomal recessive severe-to-profound hearing impairment.Human genetics · 2018Article
- Variants in CIB2 cause DFNB48 and not USH1J.Clinical genetics · 2018Article
- miR-183/96 plays a pivotal regulatory role in mouse photoreceptor maturation and maintenance.Proceedings of the National Academy of Sciences of the United States of America · 2017Article
- Maturation arrest in early postnatal sensory receptors by deletion of the miR-183/96/182 cluster in mouse.Proceedings of the National Academy of Sciences of the United States of America · 2017Article
- The statistics of the vestibular input experienced during natural self-motion differ between rodents and primates.The Journal of physiology · 2017Article
- Psychophysical testing in rodent models of glaucomatous optic neuropathy.Experimental eye research · 2015Review
- Genetics of peripheral vestibular dysfunction: lessons from mutant mouse strains.Journal of the American Academy of Audiology · 2014Review
- The mechanosensory structure of the hair cell requires clarin-1, a protein encoded by Usher syndrome III causative gene.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2012Article
- The adequate stimulus for mammalian linear vestibular evoked potentials (VsEPs).Hearing research · 2011Article
- Rotational responses of vestibular-nerve afferents innervating the semicircular canals in the C57BL/6 mouse.Journal of the Association for Research in Otolaryngology : JARO · 2008Article
- A mouse model with postnatal endolymphatic hydrops and hearing loss.Hearing research · 2008Article
- A new spontaneous mutation in the mouse protocadherin 15 gene.Hearing research · 2006Article
- The tip-link antigen, a protein associated with the transduction complex of sensory hair cells, is protocadherin-15.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2006Article
- Neuroanatomical evidence for segregation of nerve fibers conveying light touch and pain sensation in Eimer's organ of the mole.Proceedings of the National Academy of Sciences of the United States of America · 2006Article
- Progression of inner ear pathology in Ames waltzer mice and the role of protocadherin 15 in hair cell development.Journal of the Association for Research in Otolaryngology : JARO · 2006Article
- Inner ear proteomics of mouse models for deafness, a discovery strategy.Brain research · 2006Review
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Authors and funding
5 authors at 3 institutions in 1 country.
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Abstract
The deaf-circling Ames waltzer (av) mouse harbors a mutation in the protocadherin 15 (Pcdh15) gene and is a model for inner ear defects associated with Usher syndrome type 1F. Earlier studies showed altered cochlear hair cell morphology in young av mice. In contrast, no structural abnormality consistent with significant vestibular dysfunction in young av mice was observed. Light and scanning electron microscopic studies showed that vestibular hair cells from presumptive null alleles Pcdh15(av-Tg) and Pcdh15(av-3J) are morphologically similar to vestibular sensory cells from control littermates, suggesting that the observed phenotype in these alleles might be a result of a central, rather than peripheral, defect. In the present study, a combination of physiologic and anatomic methods was used to more thoroughly investigate the source of vestibular dysfunction in Ames waltzer mice. Analysis of vestibular evoked potentials and angular vestibulo-ocular reflexes revealed a lack of physiologic response to linear and angular acceleratory stimuli in Pcdh15 mutant mice. Optokinetic reflex function was diminished but still present in the mutant animals, suggesting that the defect is primarily peripheral in nature. These findings indicate that the mutation in Pcdh15 results in either a functional abnormality in the vestibular receptor organs or that the defects are limited to the vestibular nerve. AM1-43 dye uptake has been shown to correlate with normal transduction function in hair cells. Dye uptake was found to be dramatically reduced in Pcdh15 mutants compared to control littermates, suggesting that the mutation affects hair cell function, although structural abnormalities consistent with significant vestibular dysfunction are not apparent by light and scanning electron microscopy in the vestibular neuroepithelia of young animals.
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