ArticlePloS one2013
Mutation in the kv3.3 voltage-gated potassium channel causing spinocerebellar ataxia 13 disrupts sound-localization mechanisms.
Article in PloS one, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 26 papers.
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Who cites it
26 citing papers in PubMed.
- Molecular logic for cellular specializations that initiate the auditory parallel processing pathways.Nature communications · 2025Article
- Molecular logic for cellular specializations that initiate the auditory parallel processing pathways.bioRxiv : the preprint server for biology · 2024Article
- Altered binaural hearing in pre-ataxic and ataxic mutation carriers of spinocerebellar ataxia type 3.Cerebellum (London, England) · 2024Article
- Voltage-gated potassium channels as a potential therapeutic target for the treatment of neurological and psychiatric disorders.Frontiers in cellular neuroscience · 2024Review
- A novelFrontiers in cellular neuroscience · 2024Article
- Low-voltage Activating KExperimental neurobiology · 2022Article
- A missense mutation inProceedings of the National Academy of Sciences of the United States of America · 2022Article
- Article
- Multiple Roles of Actin in Exo- and Endocytosis.Frontiers in synaptic neuroscience · 2022Review
- Suppression of Kv3.3 channels by antisense oligonucleotides reverses biochemical effects and motor impairment in spinocerebellar ataxia type 13 mice.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2021Article
- Cerebellar Kv3.3 potassium channels activate TANK-binding kinase 1 to regulate trafficking of the cell survival protein Hax-1.Nature communications · 2021Article
- Presynaptic Kv3 channels are required for fast and slow endocytosis of synaptic vesicles.Neuron · 2021Article
- Modulation of Neuronal Potassium Channels During Auditory Processing.Frontiers in neuroscience · 2021Review
- Article
- Spatial Mechanisms for Segregation of Competing Sounds, and a Breakdown in Spatial Hearing.Frontiers in neuroscience · 2020Review
- Kv3 Channels: Enablers of Rapid Firing, Neurotransmitter Release, and Neuronal Endurance.Physiological reviews · 2017Review
- Physiology and anatomy of neurons in the medial superior olive of the mouse.Journal of neurophysiology · 2016Article
- Hearing and dementia.Journal of neurology · 2016Review
- Kv3.3 potassium channels and spinocerebellar ataxia.The Journal of physiology · 2016Review
- Spatial Stream Segregation by Cats.Journal of the Association for Research in Otolaryngology : JARO · 2016Article
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Authors and funding
8 authors.
Funding
Abstract
Normal sound localization requires precise comparisons of sound timing and pressure levels between the two ears. The primary localization cues are interaural time differences, ITD, and interaural level differences, ILD. Voltage-gated potassium channels, including Kv3.3, are highly expressed in the auditory brainstem and are thought to underlie the exquisite temporal precision and rapid spike rates that characterize brainstem binaural pathways. An autosomal dominant mutation in the gene encoding Kv3.3 has been demonstrated in a large Filipino kindred manifesting as spinocerebellar ataxia type 13 (SCA13). This kindred provides a rare opportunity to test in vivo the importance of a specific channel subunit for human hearing. Here, we demonstrate psychophysically that individuals with the mutant allele exhibit profound deficits in both ITD and ILD sensitivity, despite showing no obvious impairment in pure-tone sensitivity with either ear. Surprisingly, several individuals exhibited the auditory deficits even though they were pre-symptomatic for SCA13. We would expect that impairments of binaural processing as great as those observed in this family would result in prominent deficits in localization of sound sources and in loss of the "spatial release from masking" that aids in understanding speech in the presence of competing sounds.
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