ArticleInvestigative ophthalmology & visual science2006
Functional characterization of a naturally occurring Cx50 truncation.
Article in Investigative ophthalmology & visual science, 2006. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers.
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Who cites it
39 citing papers in PubMed, 51 citations in OpenAlex.
- Reversible lipid-mediated pH-gating of connexin-46/50 by cryo-EM.Nature communications · 2026Article
- Studies on Hydrostatic Pressure Responses to Piezo1 and TRPV4 in the Mouse Lens.Investigative ophthalmology & visual science · 2025Article
- The Role of Gap Junctions Dysfunction in the Development of Cataracts: From Loss of Cell-to-Cell Transfer to Blurred Vision-Review.Bioelectricity · 2023Review
- Study of The Molecular Nature of Congenital Cataracts in Patients from The Volga-Ural Region.Current issues in molecular biology · 2023Article
- Beyond the Channels: Adhesion Functions of Aquaporin 0 and Connexin 50 in Lens Development.Frontiers in cell and developmental biology · 2022Review
- Loss of fiber cell communication may contribute to the development of cataracts of many different etiologies.Frontiers in physiology · 2022Review
- Proposed Role for Internal Lens Pressure as an Initiator of Age-Related Lens Protein Aggregation Diseases.Clinical ophthalmology (Auckland, N.Z.) · 2022Article
- Article
- Connexin Mutants Compromise the Lens Circulation and Cause Cataracts through Biomineralization.International journal of molecular sciences · 2020Review
- Signaling Between TRPV1/TRPV4 and Intracellular Hydrostatic Pressure in the Mouse Lens.Investigative ophthalmology & visual science · 2020Article
- GJA8 missense mutation disrupts hemichannels and induces cell apoptosis in human lens epithelial cells.Scientific reports · 2019Article
- Electrical coupling and its channels.The Journal of general physiology · 2018Review
- Acetylation of C-terminal lysines modulates protein turnover and stability of Connexin-32.BMC cell biology · 2018Article
- Aquaporin 0 Modulates Lens Gap Junctions in the Presence of Lens-Specific Beaded Filament Proteins.Investigative ophthalmology & visual science · 2017Article
- Focus on lens connexins.BMC cell biology · 2017Review
- Identification and Functional Assessment of Age-Dependent Truncations to Cx46 and Cx50 in the Human Lens.Investigative ophthalmology & visual science · 2016Article
- The E368Q Mutant Allele ofOpen access journal of ophthalmology · 2016Article
- Mutant connexin 50 (S276F) inhibits channel and hemichannel functions inducing cataract.Journal of genetics · 2015Article
- Lens ion homeostasis relies on the assembly and/or stability of large connexin 46 gap junction plaques on the broad sides of differentiating fiber cells.American journal of physiology. Cell physiology · 2015Article
- A connexin50 mutant, CX50fs, that causes cataracts is unstable, but is rescued by a proteasomal inhibitor.The Journal of biological chemistry · 2013Article
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
Abstract
purposeLens connexins undergo proteolytic cleavage of their C termini during fiber maturation. Although the functional significance of this is unknown, cleavage has been correlated with changes in channel-gating properties. This study evaluates the functional consequences of this endogenous truncation by characterizing the properties of a C-terminal truncated Cx50 protein.
methodsMurine and human Cx50 were truncated at amino acids 290 and 294, respectively, before expression in paired Xenopus oocytes or mammalian cells. Protein expression was evaluated by immunocytochemistry. Dual whole-cell voltage clamp techniques were used to analyze macroscopic and single-channel conductance, voltage-gating properties, and kinetics; pH gating sensitivity was measured by superfusion with 100% CO2-saturated media.
resultsCx50tr290 channels exhibited an 86% to 89% reduction in mean macroscopic conductance compared with full-length Cx50. Heterotypic channels formed functional gap junctions, displayed an intermediate level of coupling, and exhibited unaltered voltage-gating properties. C-terminal truncation did not alter single-channel gating characteristics or unitary conductance. Interestingly, truncated and full-length Cx50 channel conductances were reversibly blocked by cytoplasmic acidification.
conclusionsC-terminal truncation of Cx50 did not inhibit the formation of homotypic or heterotypic channels. However, a significant decrease in conductance was observed for truncated channels, a phenomenon independent of alterations in voltage-gating sensitivity, kinetics, or chemical gating. These results provide a plausible explanation for the 50% decrease in junctional coupling observed during lens fiber maturation.
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