ArticleJournal of the Association for Research in Otolaryngology : JARO2004
Null mutation of alpha1D Ca2+ channel gene results in deafness but no vestibular defect in mice.
Article in Journal of the Association for Research in Otolaryngology : JARO, 2004. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 76 papers.
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Who cites it
76 citing papers in PubMed, 120 citations in OpenAlex.
- Multi-omics analysis of ion channels expressed by chicken embryo vestibular type I and type II hair cells.Experimental physiology · 2026Article
- Molecular basis for a CaThe Journal of biological chemistry · 2026Article
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- Hair cell loss - cause or consequence of hearing loss?Frontiers in cell and developmental biology · 2026Review
- Genetic and pharmacologic alterations of claudin9 levels suffice to induce functional and mature inner hair cells.bioRxiv : the preprint server for biology · 2024Article
- Demyelination and NaeNeuro · 2024Article
- mTORC2 regulates auditory hair cell structure and function.iScience · 2023Article
- Multiple Calcium Channel Types with Unique Expression Patterns Mediate Retinal Signaling at Bipolar Cell Ribbon Synapses.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2022Article
- Signal transmission in mature mammalian vestibular hair cells.Frontiers in cellular neuroscience · 2022Article
- Rbm24 regulates inner-ear-specific alternative splicing and is essential for maintaining auditory and motor coordination.RNA biology · 2021Article
- Current Response in CaFrontiers in neuroscience · 2021Article
- Exocytosis in mouse vestibular Type II hair cells shows a high-order CaPhysiological reports · 2020Article
- Distribution and Functional Characteristics of Voltage-Gated Sodium Channels in Immature Cochlear Hair Cells.Neuroscience bulletin · 2020Article
- Recent advances in cochlear hair cell nanophysiology: subcellular compartmentalization of electrical signaling in compact sensory cells.Faculty reviews · 2020Review
- The local translation ofAging · 2019Article
- Article
- Pou4f1 Defines a Subgroup of Type I Spiral Ganglion Neurons and Is Necessary for Normal Inner Hair Cell Presynaptic CaThe Journal of neuroscience : the official journal of the Society for Neuroscience · 2019Article
- Nanomachinery Organizing Release at Neuronal and Ribbon Synapses.International journal of molecular sciences · 2019Review
- Intrinsic planar polarity mechanisms influence the position-dependent regulation of synapse properties in inner hair cells.Proceedings of the National Academy of Sciences of the United States of America · 2019Article
- Cochlea-Specific Deletion of CaFrontiers in cellular neuroscience · 2019Article
16 more citing papers are in PubMed but not listed here.
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Authors and funding
9 authors at 4 institutions in 3 countries.
Funding
Abstract
Multiple Ca2+ channels confer diverse functions to hair cells of the auditory and vestibular organs in the mammalian inner ear. We used gene-targeting technology to generate alpha1D Ca2+ channel-deficient mice to determine the physiological role of these Ca2+ channels in hearing and balance. Analyses of auditory-evoked brainstem recordings confirmed that alpha1D-/- mice were deaf and revealed that heterozygous (alpha1D+/-) mice have increased hearing thresholds. However, hearing deficits in alpha1D+/- mice were manifested mainly by the increase in threshold of low-frequency sounds. In contrast to impaired hearing, alpha1D-/- mice have balance performances equivalent to their wild-type littermates. Light and electron microscope analyses of the inner ear revealed outer hair cell loss at the apical cochlea, but no apparent abnormality at the basal cochlea and the vestibule. We determined the mechanisms underlying the auditory function defects and the normal vestibular functions by examining the Ba2+ currents in cochlear inner and outer hair cells versus utricular hair cells in alpha1D+/- mice. Whereas the whole-cell Ba2+ currents in inner hair cells consist mainly of the nimodipine-sensitive current (approximately 85%), the utricular hair cells express only approximately 50% of this channel subtype. Thus, differential expression of alpha1D channels in the cochlear and utricular hair cells confers the phenotype of the alpha1D null mutant mice. Because vestibular and cochlear hair cells share common features and null deletion of several genes have yielded both deafness and imbalance in mice, alpha1D null mutant mice may serve as a model to disentangle vestibular from auditory-specific functions.
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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.