ArticleThe Journal of clinical investigation2013
Tricellulin deficiency affects tight junction architecture and cochlear hair cells.
Article in The Journal of clinical investigation, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 59 papers.
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Who cites it
59 citing papers in PubMed, 100 citations in OpenAlex.
- Roles of protein-protein interactions and monolayer mechanics in tricellulin localization to tricellular tight junctions.Biology open · 2025Article
- A feedback circuitry involving γ-actin, β-actin and nonmuscle myosin-2 A controls tight junction and apical cortex mechanics.Nature communications · 2025Article
- Novel compound heterozygous variants in MARVELD2 causing autosomal recessive hearing loss in two Chinese families.Molecular genetics & genomic medicine · 2024Article
- A short guide to the tight junction.Journal of cell science · 2024Review
- Autosomal recessive non-syndromic hearing loss genes in Pakistan during the previous three decades.Journal of cellular and molecular medicine · 2024Review
- Case report: A novel nonsense mutation in theFrontiers in genetics · 2024Article
- Cingulin regulates hair cell cuticular plate morphology and is required for hearing in human and mouse.EMBO molecular medicine · 2023Article
- Combined AAV-mediated gene replacement therapy improves auditory function in a mouse model of human DFNB42 deafness.Molecular therapy : the journal of the American Society of Gene Therapy · 2023Article
- β-Catenin transcriptional activity is required for establishment of inner pillar cell identity during cochlear development.PLoS genetics · 2023Article
- Precise detection of CRISPR-Cas9 editing in hair cells in the treatment of autosomal dominant hearing loss.Molecular therapy. Nucleic acids · 2022Article
- Local Delivery of Therapeutics to the Cochlea Using Nanoparticles and Other Biomaterials.Pharmaceuticals (Basel, Switzerland) · 2022Review
- Tricellulin secures the epithelial barrier at tricellular junctions by interacting with actomyosin.The Journal of cell biology · 2022Article
- Presentation of potential genes and deleterious variants associated with non-syndromic hearing loss: a computational approach.Genomics & informatics · 2022Article
- Angulin-1 seals tricellular contacts independently of tricellulin and claudins.The Journal of cell biology · 2021Article
- Brain endothelial tricellular junctions as novel sites for T cell diapedesis across the blood-brain barrier.Journal of cell science · 2021Article
- The Role of Tricellular Junctions in the Transport of Macromolecules Across Endothelium.Cardiovascular engineering and technology · 2021Review
- In vivo base editing restores sensory transduction and transiently improves auditory function in a mouse model of recessive deafness.Science translational medicine · 2020Article
- The extracellular domain of angulin-1 and palmitoylation of its cytoplasmic region are required for angulin-1 assembly at tricellular contacts.The Journal of biological chemistry · 2020Article
- The multifarious regulation of the apical junctional complex.Open biology · 2020Review
- Growth factor and receptor malfunctions associated with human genetic deafness.Clinical genetics · 2020Review
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Authors and funding
14 authors at 6 institutions in 3 countries.
Funding
Abstract
The two compositionally distinct extracellular cochlear fluids, endolymph and perilymph, are separated by tight junctions that outline the scala media and reticular lamina. Mutations in TRIC (also known as MARVELD2), which encodes a tricellular tight junction protein known as tricellulin, lead to nonsyndromic hearing loss (DFNB49). We generated a knockin mouse that carries a mutation orthologous to the TRIC coding mutation linked to DFNB49 hearing loss in humans. Tricellulin was absent from the tricellular junctions in the inner ear epithelia of the mutant animals, which developed rapidly progressing hearing loss accompanied by loss of mechanosensory cochlear hair cells, while the endocochlear potential and paracellular permeability of a biotin-based tracer in the stria vascularis were unaltered. Freeze-fracture electron microscopy revealed disruption of the strands of intramembrane particles connecting bicellular and tricellular junctions in the inner ear epithelia of tricellulin-deficient mice. These ultrastructural changes may selectively affect the paracellular permeability of ions or small molecules, resulting in a toxic microenvironment for cochlear hair cells. Consistent with this hypothesis, hair cell loss was rescued in tricellulin-deficient mice when generation of normal endolymph was inhibited by a concomitant deletion of the transcription factor, Pou3f4. Finally, comprehensive phenotypic screening showed a broader pathological phenotype in the mutant mice, which highlights the non-redundant roles played by tricellulin.
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