ReviewCold Spring Harbor perspectives in medicine2019
Inner Ear Connexin Channels: Roles in Development and Maintenance of Cochlear Function.
Review in Cold Spring Harbor perspectives in medicine, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 43 papers.
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Who cites it
43 citing papers in PubMed, 65 citations in OpenAlex.
- Clinical and Genetic Characterization of Gap Junction Protein β-6 Variants in Non-Syndromic Hearing Loss: A Case Report with Familial Evaluation andIranian journal of medical sciences · 2026Article
- Genotype-guided Recall Delineates the Adult Auditory Phenotype in GJB2 p.V37I Homozygotes: High-frequency Vulnerability and Environmental Modulation.Otology & neurotology : official publication of the American Otological Society, American Neurotology Society [and] European Academy of Otology and Neurotology · 2026Article
- Connexin 26 in Hearing Health and Disease: StructuralFoundations, Mutation Mechanisms, and Therapeutic Perspectives.International journal of molecular sciences · 2026Review
- Recent Progress in Mechanism-Based Therapies forInternational journal of molecular sciences · 2026Review
- E114G de Novo Mutation in GJB2 Gene in a Chinese Patient with Classical Vohwinkel Syndrome.International medical case reports journal · 2026Article
- Article
- Cochlear endoplasmic reticulum stress causes connexin 26 degradation is involved in age-related hearing loss.iScience · 2025Article
- Cell-specific delivery of GJB2 restores auditory function in mouse models of DFNB1 deafness and mediates appropriate expression in NHP cochlea.Nature communications · 2025Article
- A rare haplotype of the GJD3 gene segregating in familial Meniere's disease interferes with connexin assembly.Genome medicine · 2025Article
- Gap junctions in Turing-type periodic feather pattern formation.PLoS biology · 2024Article
- Optogenetics Reveals Roles for Supporting Cells in Force Transmission to and From Outer Hair Cells in the Mouse Cochlea.The Journal of neuroscience : the official journal of the Society for Neuroscience · 2024Article
- Oxidative stress and inflammation cause auditory system damage via glial cell activation and dysregulated expression of gap junction proteins in an experimental model of styrene-induced oto/neurotoxicity.Journal of neuroinflammation · 2024Article
- GJB2 c.35del variant up-regulates GJA1 gene expression and affects differentiation of human stem cells.Genetics and molecular biology · 2024Article
- Functional Consequences of Pathogenic Variants of theBiomolecules · 2023Review
- Divergence between Hemichannel and Gap Junction Permeabilities of Connexin 30 and 26.Life (Basel, Switzerland) · 2023Article
- Hereditary deafness carrier screening in 9,993 Chinese individuals.Frontiers in genetics · 2023Article
- Non-Syndromic Hearing Loss in a Romanian Population: Carrier Status and Frequent Variants in theGenes · 2022Article
- Article
- Connexin Mutations and Hereditary Diseases.International journal of molecular sciences · 2022Review
- The Interplay of Cx26, Cx32, Cx37, Cx40, Cx43, Cx45, and Panx1 in Inner-Ear Development of Yotari (dab1-/-) Mice and Humans.Biomedicines · 2022Article
Corrections and comments
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Authors and funding
1 author at 1 institution in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Connexin 26 and connexin 30 are the prevailing isoforms in the epithelial and connective tissue gap junction systems of the developing and mature cochlea. The most frequently encountered variants of the genes that encode these connexins, which are transcriptionally coregulated, determine complete loss of protein function and are the predominant cause of prelingual hereditary deafness. Reducing connexin 26 expression by Cre/loxP recombination in the inner ear of adult mice results in a decreased endocochlear potential, increased hearing thresholds, and loss of >90% of outer hair cells, indicating that this connexin is essential for maintenance of cochlear function. In the developing cochlea, connexins are necessary for intercellular calcium signaling activity. Ribbon synapses and basolateral membrane currents fail to mature in inner hair cells of mice that are born with reduced connexin expression, even though hair cells do not express any connexin. In contrast, pannexin 1, an alternative mediator of intercellular signaling, is dispensable for hearing acquisition and auditory function.
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