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A fully human IgG1 antibody targeting connexin 32 extracellular domain blocks CMTX1 hemichannel dysfunction in an in vitro model.
Cell Commun Signal
December 2024
CNR Institute of Biochemistry and Cell Biology, Monterotondo, Rome, 00015, Italy.
Connexins (Cxs) are fundamental in cell-cell communication, functioning as gap junction channels (GJCs) that facilitate solute exchange between adjacent cells and as hemichannels (HCs) that mediate solute exchange between the cytoplasm and the extracellular environment. Mutations in the GJB1 gene, which encodes Cx32, lead to X-linked Charcot-Marie-Tooth type 1 (CMTX1), a rare hereditary demyelinating disorder of the peripheral nervous system (PNS) without an effective cure or treatment. In Schwann cells, Cx32 HCs are thought to play a role in myelination by enhancing intracellular and intercellular Ca signaling, which is crucial for proper PNS myelination.
View Article and Find Full Text PDFMolecular Dynamics Simulation of Permeation Through Connexin Channels.
Methods Mol Biol
April 2024
Institut Pasteur de Montevideo, Montevideo, Uruguay.
Molecular dynamics (MD) simulations are a collection of computational tools that can be used to trace intermolecular interactions at the sub-nanometer level. They offer possibilities that are often unavailable to experimental methods, making MD an ideal complementary technique for the understanding a plethora of biological processes. Thanks to significant efforts by many groups of developers around the world, setting up and running MD simulations has become progressively simpler.
View Article and Find Full Text PDFConnexons Coupling to Gap Junction Channel: Potential Role for Extracellular Protein Stabilization Centers.
Biomolecules
December 2021
Research Centre for Natural Sciences, Institute of Organic Chemistry, Magyar Tudósok Körútja 2, 1117 Budapest, Hungary.
Connexin (Cx) proteins establish intercellular gap junction channels (Cx GJCs) through coupling of two apposed hexameric Cx hemichannels (Cx HCs, connexons). Pre- and post-GJ interfaces consist of extracellular EL1 and EL2 loops, each with three conserved cysteines. Previously, we reported that known peptide inhibitors, mimicking a variety of Cx43 sequences, appear non-selective when binding to homomeric Cx43 vs.
View Article and Find Full Text PDFMaking connexons in the neurovascular unit.
J Cereb Blood Flow Metab
August 2012
Department of Biomedical Sciences and Brain Barriers Research Center, University of Minnesota Medical School Duluth, Duluth, Minnesota, USA.
Structural models of the supramolecular organization of AQP0 and connexons in junctional microdomains.
J Struct Biol
December 2007
Institut Curie, UMR168-CNRS, 26 Rue d'Ulm, 75248 Paris, France.
Membrane proteins perform many essential cellular functions. Over the last years, substantial advances have been made in our understanding of the structure and function of isolated membrane proteins. However, like soluble proteins, many membrane proteins assemble into supramolecular complexes that perform specific functions in specialized membrane domains.
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