Gjb6

Summary

Gene Symbol: Gjb6
Description: gap junction protein, beta 6
Alias: AA958971, Cx30, D14Bwg0506e, gap junction beta-6 protein, connexin-30, gap junction membrane channel protein beta 6
Species: mouse

Top Publications

  1. pmc Connexin30 deficiency causes instrastrial fluid-blood barrier disruption within the cochlear stria vascularis
    Martine Cohen-Salmon
    Unité de Génétique des Déficits Sensoriels, Unité Mixte de Recherche S 587, Institut National de la Sante et de la Recherche Medicale, 75724 Paris Cedex 15, France
    Proc Natl Acad Sci U S A 104:6229-34. 2007
  2. pmc Astroglial networks scale synaptic activity and plasticity
    Ulrike Pannasch
    Centre National de la Recherche Scientifique Unité Mixte de Recherche 7241 Institut National de la Santé et de la Recherche Médicale U1050, College de France, 75005 Paris, France
    Proc Natl Acad Sci U S A 108:8467-72. 2011
  3. pmc Restoration of connexin26 protein level in the cochlea completely rescues hearing in a mouse model of human connexin30-linked deafness
    Shoeb Ahmad
    Department of Otolaryngology, Yerkes Microarray Core, Emory University School of Medicine, 615 Michael Street, Atlanta, GA 30322 3030, USA
    Proc Natl Acad Sci U S A 104:1337-41. 2007
  4. ncbi The impact of astrocytic gap junctional coupling on potassium buffering in the hippocampus
    Anke Wallraff
    Department of Experimental Neurobiology, Neurosurgery, University of Bonn, 53105 Bonn, Germany
    J Neurosci 26:5438-47. 2006
  5. pmc Gap junction-mediated intercellular biochemical coupling in cochlear supporting cells is required for normal cochlear functions
    Yanping Zhang
    Department of Otolaryngology, Emory University School of Medicine, 615 Michael Street, Atlanta, GA 30322 3030, USA
    Proc Natl Acad Sci U S A 102:15201-6. 2005
  6. doi Quality control of astrocyte-directed Cre transgenic mice: the benefits of a direct link between loss of gene expression and reporter activation
    Robert Pascal Requardt
    Institute of Genetics, Division of Molecular Genetics, University of Bonn, D 53117 Bonn, Germany
    Glia 57:680-92. 2009
  7. pmc ATP release through connexin hemichannels and gap junction transfer of second messengers propagate Ca2+ signals across the inner ear
    Fabio Anselmi
    Foundation for Advanced Biomedical Research, Venetian Institute of Molecular Medicine, 35129 Padua, Italy
    Proc Natl Acad Sci U S A 105:18770-5. 2008
  8. ncbi Cochlear gap junctions coassembled from Cx26 and 30 show faster intercellular Ca2+ signaling than homomeric counterparts
    Jianjun Sun
    Section on Neurobiology, Leslie and Susan Gonda Department of Cell and Molecular Biology, House Ear Institute, Los Angeles, California, USA
    Am J Physiol Cell Physiol 288:C613-23. 2005
  9. pmc Coordinated control of connexin 26 and connexin 30 at the regulatory and functional level in the inner ear
    Saida Ortolano
    Venetian Institute of Molecular Medicine, Foundation for Advanced Biomedical Research, 35129 Padua, Italy
    Proc Natl Acad Sci U S A 105:18776-81. 2008
  10. doi Astroglial metabolic networks sustain hippocampal synaptic transmission
    Nathalie Rouach
    Inserm U840, College de France, 11 place Marcelin Berthelot, 75005 Paris, France
    Science 322:1551-5. 2008

Research Grants

Scientific Experts

  • A Forge
  • Nathalie Rouach
  • James Stockand
  • Anne Dupressoir
  • Sarah E Lutz
  • K R Johnson
  • P M Kelley
  • Darren Locke
  • Jane H C Lin
  • Annette Koulakoff
  • Daniel Gros
  • Klaus Willecke
  • Martin Theis
  • Christian Giaume
  • Christian Steinhäuser
  • Xi Lin
  • Martine Cohen-Salmon
  • Wenxue Tang
  • Lisa Roux
  • Gerald Seifert
  • Ulrike Pannasch
  • Pascal Ezan
  • Fabio Mammano
  • Giulia Crispino
  • Julia Langer
  • Jin Chen
  • Yan Qu
  • Qing Chang
  • Jiong Zhang
  • Oliver Tress
  • Joachim Degen
  • Goran Sohl
  • Pavel Dublin
  • Fabio Anselmi
  • Veronica Abudara
  • Shoeb Ahmad
  • Christine Petit
  • Karim Benchenane
  • Dennis May
  • Anne Cécile Boulay
  • Peter Bedner
  • Laura Rodriguez
  • Ping Chen
  • Francisco J del Castillo
  • Shoab Ahmad
  • Saida Ortolano
  • Stuart L Johnson
  • Jette Skov Alstrøm
  • Stephanie Griemsmann
  • Hong Bo Zhao
  • Chun Liang
  • Glenn Dallérac
  • Jing Chen
  • Yan Zhu
  • Christine R Rose
  • Amy C Berger
  • Kazusaku Kamiya
  • Jérémie Sibille
  • Nadine Richter
  • Helmut Kettenmann
  • Ronald Jabs
  • Marie Liebmann
  • Xinhe Liu
  • Atsushi Fujimoto
  • Nicola G Davis
  • Irina V Majoul
  • David Holcman
  • Michael V L Bennett
  • Yanping Zhang
  • Tina Unger
  • Weiguo Peng
  • Dale W Laird
  • Michael J Chen
  • Chunlan Huang
  • Xiaoning Han
  • Qing Shao
  • Maiken Nedergaard
  • Markus Kretz
  • Jianjun Sun
  • Mario Bortolozzi
  • Pietro Scimemi
  • Elena Mironova
  • Paromita Majumder
  • Carl Ernst
  • B D Lynn
  • Dominic Gosejacob
  • K Willecke
  • Binfei Zhou
  • Isabelle Plante
  • Kerstin Hüttmann

Detail Information

Publications77

  1. pmc Connexin30 deficiency causes instrastrial fluid-blood barrier disruption within the cochlear stria vascularis
    Martine Cohen-Salmon
    Unité de Génétique des Déficits Sensoriels, Unité Mixte de Recherche S 587, Institut National de la Sante et de la Recherche Medicale, 75724 Paris Cedex 15, France
    Proc Natl Acad Sci U S A 104:6229-34. 2007
    ..The absence of the gap junction protein connexin30 (Cx30) in Cx30(-/-) mice results in the SV failure to produce an EP, which mainly accounts for the severe congenital ..
  2. pmc Astroglial networks scale synaptic activity and plasticity
    Ulrike Pannasch
    Centre National de la Recherche Scientifique Unité Mixte de Recherche 7241 Institut National de la Santé et de la Recherche Médicale U1050, College de France, 75005 Paris, France
    Proc Natl Acad Sci U S A 108:8467-72. 2011
    ..Although the gap junction proteins connexin 30 (Cx30) and connexin 43 (Cx43) mediate the extensive network organization of astrocytes, their role in synaptic ..
  3. pmc Restoration of connexin26 protein level in the cochlea completely rescues hearing in a mouse model of human connexin30-linked deafness
    Shoeb Ahmad
    Department of Otolaryngology, Yerkes Microarray Core, Emory University School of Medicine, 615 Michael Street, Atlanta, GA 30322 3030, USA
    Proc Natl Acad Sci U S A 104:1337-41. 2007
    Mutations in genes coding for connexin26 (Cx26) and/or Cx30 are linked to approximately half of all cases of human autosomal nonsyndromic prelingual deafness...
  4. ncbi The impact of astrocytic gap junctional coupling on potassium buffering in the hippocampus
    Anke Wallraff
    Department of Experimental Neurobiology, Neurosurgery, University of Bonn, 53105 Bonn, Germany
    J Neurosci 26:5438-47. 2006
    ..In general, a surprisingly large capacity for K+ clearance was conserved in mice with coupling-deficient astrocytes, indicating that gap junction-dependent processes only partially account for K+ buffering in the hippocampus...
  5. pmc Gap junction-mediated intercellular biochemical coupling in cochlear supporting cells is required for normal cochlear functions
    Yanping Zhang
    Department of Otolaryngology, Emory University School of Medicine, 615 Michael Street, Atlanta, GA 30322 3030, USA
    Proc Natl Acad Sci U S A 102:15201-6. 2005
    Dysfunction of gap junctions (GJs) caused by mutations in connexin26 (Cx26) and Cx30 accounts for nearly half of all cases of hereditary nonsyndromic deafness cases...
  6. doi Quality control of astrocyte-directed Cre transgenic mice: the benefits of a direct link between loss of gene expression and reporter activation
    Robert Pascal Requardt
    Institute of Genetics, Division of Molecular Genetics, University of Bonn, D 53117 Bonn, Germany
    Glia 57:680-92. 2009
    ..We present several strategies to control for the fidelity of hGFAP-Cre mediated recombination. (c) 2008 Wiley-Liss, Inc...
  7. pmc ATP release through connexin hemichannels and gap junction transfer of second messengers propagate Ca2+ signals across the inner ear
    Fabio Anselmi
    Foundation for Advanced Biomedical Research, Venetian Institute of Molecular Medicine, 35129 Padua, Italy
    Proc Natl Acad Sci U S A 105:18770-5. 2008
    ..By contrast, Ca(2+) responses failed to propagate in cultures with defective expression of connexin 26 (Cx26) or Cx30. A companion paper demonstrates that, if expression of either Cx26 or Cx30 is blocked, expression of the other is ..
  8. ncbi Cochlear gap junctions coassembled from Cx26 and 30 show faster intercellular Ca2+ signaling than homomeric counterparts
    Jianjun Sun
    Section on Neurobiology, Leslie and Susan Gonda Department of Cell and Molecular Biology, House Ear Institute, Los Angeles, California, USA
    Am J Physiol Cell Physiol 288:C613-23. 2005
    ..However, it is still unclear how Cxs contribute to the cochlear function. Recent data (33) obtained from Cx30 knockout mice showing that a reduction of Cx diversity in assembling gap junctions is sufficient to cause deafness ..
  9. pmc Coordinated control of connexin 26 and connexin 30 at the regulatory and functional level in the inner ear
    Saida Ortolano
    Venetian Institute of Molecular Medicine, Foundation for Advanced Biomedical Research, 35129 Padua, Italy
    Proc Natl Acad Sci U S A 105:18776-81. 2008
    Connexin 26 (Cx26) and connexin 30 (Cx30) are encoded by two genes (GJB2 and GJB6, respectively) that are found within 50 kb in the same complex deafness locus, DFNB1...
  10. doi Astroglial metabolic networks sustain hippocampal synaptic transmission
    Nathalie Rouach
    Inserm U840, College de France, 11 place Marcelin Berthelot, 75005 Paris, France
    Science 322:1551-5. 2008
    ..These results indicate that astroglial gap junctions provide an activity-dependent intercellular pathway for the delivery of energetic metabolites from blood vessels to distal neurons...
  11. ncbi Gap junctions in the inner ear: comparison of distribution patterns in different vertebrates and assessement of connexin composition in mammals
    Andrew Forge
    UCL Centre for Auditory Research and Institute of Laryngology and Otology, University College London, London WC1X 8EE, United Kingdom
    J Comp Neurol 467:207-31. 2003
    ..mammalian inner ear by RT-PCR, Western blotting, and immunohistochemistry revealed four connexin isotypes, cx26, cx30, cx31, and cx43, in the cochlea and three, cx26, cx30, and cx43, in the vestibular organs...
  12. ncbi Connexins 26 and 30 are co-assembled to form gap junctions in the cochlea of mice
    Shoab Ahmad
    Section on Neurobiology, Leslie and Susan Gonda Department of Cell and Molecular Biology, House Ear Institute, Los Angeles, CA 90057 1922, USA
    Biochem Biophys Res Commun 307:362-8. 2003
    ..Two of these connexins (Cx26 and Cx30), both belonging to the beta-group, were investigated for their molecular assemblies in the cochlea...
  13. pmc Connexin expression by radial glia-like cells is required for neurogenesis in the adult dentate gyrus
    Albrecht Kunze
    Institute of Cellular Neurosciences, Medical Faculty, University of Bonn, Sigmund Freud Strasse 25, 53105 Bonn, Germany
    Proc Natl Acad Sci U S A 106:11336-41. 2009
    ..Neuronal precursors were not coupled. Mice lacking Cx30 and Cx43 in GFAP-positive cells displayed almost complete inhibition of proliferation and a significant decline in ..
  14. pmc Connexin30 null and conditional connexin26 null mice display distinct pattern and time course of cellular degeneration in the cochlea
    Yu Sun
    Department of Otolaryngology, Emory University School of Medicine, Atlanta, GA 30322 3030, USA
    J Comp Neurol 516:569-79. 2009
    Mutations in connexin26 (Cx26) and Cx30 are the most common cause of nonsyndromic inherited deafness in humans...
  15. doi Role of astroglial connexin30 in hippocampal gap junction coupling
    Dominic Gosejacob
    Institute of Cellular Neurosciences, Medical Faculty, University of Bonn, 53105 Bonn, Germany
    Glia 59:511-9. 2011
    The impact of connexin30 (Cx30) on interastrocytic gap junction coupling in the normal hippocampus is matter of debate; reporter gene analyses indicated a weak expression of Cx30 in the mouse hippocampus...
  16. ncbi Connexin30 (Gjb6)-deficiency causes severe hearing impairment and lack of endocochlear potential
    Barbara Teubner
    Institut fur Genetik, Abteilung Molekulargenetik, Universitat Bonn, Bonn, Germany
    Hum Mol Genet 12:13-21. 2003
    The gap junction protein connexin30 (Cx30) is expressed in a variety of tissues that include epithelial and mesenchymal structures of the inner ear. We generated Cx30 (Gjb6) deficient mice by deletion of the Cx30 coding region...
  17. doi Astrocytic Cx43 and Cx30 differentially modulate adult neurogenesis in mice
    Marie Liebmann
    Hans Berger Department of Neurology, Jena University Hospital, Erlanger Allee 101, 07747 Jena, Germany
    Neurosci Lett 545:40-5. 2013
    ..The aim of this study was to determine whether loss of the main astrocytic connexins, connexin 43 (Cx43) or connexin 30 (Cx30), influences various stages of adult hippocampal neurogenesis...
  18. doi Connexin30-mediated intercellular communication plays an essential role in epithelial repair in the cochlea
    Andrew Forge
    Centre for Auditory Research, UCL Ear Institute, London WC1X 8EE, UK
    J Cell Sci 126:1703-12. 2013
    ..Gap junctions in which Cx30 is the predominant connexin are large and numerous between supporting cells...
  19. doi Connexin dynamics in the privileged wound healing of the buccal mucosa
    Nicola G Davis
    Department of Cell and Developmental Biology, University College London, London, United Kingdom
    Wound Repair Regen 21:571-8. 2013
    ..This rapid down-regulation of all three connexins may in part underlie the rapid healing of the buccal mucosa. ..
  20. doi The psychostimulant modafinil enhances gap junctional communication in cortical astrocytes
    Xinhe Liu
    College de France, Center for Interdisciplinary Research in Biology CIRB, Centre National de la Recherche Scientifique, Unité Mixte de Recherche 7241, Institut National de la Santé et de la Recherche Médicale Unité 1050, 75231 Paris Cedex 05, France University Pierre et Marie Curie, ED, n 158, 75005 Paris, France MEMOLIFE Laboratory of Excellence and Paris Science Lettre Research University, 75005 Paris, France
    Neuropharmacology 75:533-8. 2013
    ..We report that in the cortex modafinil injection increases the expression of mRNA and protein of connexin 30 but not those of connexin 43, the other major astroglial connexin...
  21. pmc Connexin-Mediated Signaling in Nonsensory Cells Is Crucial for the Development of Sensory Inner Hair Cells in the Mouse Cochlea
    Stuart L Johnson
    Department of Biomedical Science, University of Sheffield, S10 2TN Sheffield, United Kingdom
    J Neurosci 37:258-268. 2017
    Mutations in the genes encoding for gap junction proteins connexin 26 (Cx26) and connexin 30 (Cx30) have been linked to syndromic and nonsyndromic hearing loss in mice and humans...
  22. pmc Astroglial potassium clearance contributes to short-term plasticity of synaptically evoked currents at the tripartite synapse
    Jérémie Sibille
    N Rouach Neuroglial Interactions in Cerebral Physiopathology, College de France, CIRB, CNRS UMR 7241, INSERM U1050, 11, place Marcelin Berthelot, 75005 Paris, France
    J Physiol 592:87-102. 2014
    ..These results show that astrocytes integrate synaptic activity via multiple ionic channels and transporters and contribute to short-term plasticity in part via potassium clearance mediated by Kir4.1 channels...
  23. doi Mutations in Cx30 that are linked to skin disease and non-syndromic hearing loss exhibit several distinct cellular pathologies
    Amy C Berger
    Department of Physiology and Pharmacology, University of Western Ontario, London, ON N6A 5C1, Canada
    J Cell Sci 127:1751-64. 2014
    b>Connexin 30 (Cx30), a member of the large gap-junction protein family, plays a role in the homeostasis of the epidermis and inner ear through gap junctional intercellular communication (GJIC)...
  24. doi Rapid sodium signaling couples glutamate uptake to breakdown of ATP in perivascular astrocyte endfeet
    Julia Langer
    Faculty of Mathematics and Natural Sciences, Institute of Neurobiology, Heinrich Heine University Duesseldorf, Universitatsstrasse 1, Duesseldorf, D 40225, Germany
    Glia 65:293-308. 2017
    ..of ∼120 µm/s and directly propagated from stimulated into neighboring endfeet; this spread was omitted in Cx30/Cx43 double-deficient mice...
  25. doi Astroglial Connexin 43 Hemichannels Modulate Olfactory Bulb Slow Oscillations
    Lisa Roux
    Center for Interdisciplinary Research in Biology, Collège de France Centre National de la Recherche Scientifique, Unité Mixte de Recherche 7241, Institut National de la Santé et de la Recherche Médicale U1050, 75231 Paris Cedex 05, France, University Pierre et Marie Curie, 75005 Paris, France, MemoLife Laboratory of Excellence and Paris Science Lettre Research University, 75005 Paris, France
    J Neurosci 35:15339-52. 2015
    ..These results highlight a new mechanism of neuroglial interaction in the olfactory bulb, where astrocyte connexin hemichannels are both targets and modulators of neuronal circuit function...
  26. pmc Isoform-specific phosphorylation-dependent regulation of connexin hemichannels
    Jette Skov Alstrøm
    Department of Cellular and Molecular Medicine, Faculty of Health and Medical Sciences, University of Copenhagen, Copenhagen, Denmark and
    J Neurophysiol 114:3014-22. 2015
    ..Connexin (Cx)43 and Cx30 are the major astrocytic connexins...
  27. pmc Pannexin1 channels dominate ATP release in the cochlea ensuring endocochlear potential and auditory receptor potential generation and hearing
    Jin Chen
    1 Department of Otolaryngology, University of Kentucky Medical Center, 800 Rose Street, Lexington, Kentucky, 40536, USA 2 Department of Otolaryngology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, P R of China
    Sci Rep 5:10762. 2015
    ..Moreover, defect of connexin hemichannels by deletion of connexin26 (Cx26) and Cx30, which are predominant connexin isoforms in the cochlea, did not reduce ATP release under physiological conditions...
  28. doi Activated microglia impairs neuroglial interaction by opening Cx43 hemichannels in hippocampal astrocytes
    Veronica Abudara
    College de France, Center for Interdisciplinary Research in Biology CIRB Centre National de la Recherche Scientifique, Unité Mixte de Recherche 7241 Institut National de la Santé et de la Recherche Médicale U1050, Paris Cedex, France University Pierre et Marie Curie, ED, Paris, France MEMOLIFE Laboratory of Excellence and Paris Science Lettre Research University, Paris, France Departamento de Fisiología, Facultad de Medicina, Universidad de la Republica, Montevideo, Uruguay
    Glia 63:795-811. 2015
    ..As a whole our findings point out astroglial hemichannels as key determinants of the impairment of synaptic transmission during neuroinflammation...
  29. pmc Characterization of Panglial Gap Junction Networks in the Thalamus, Neocortex, and Hippocampus Reveals a Unique Population of Glial Cells
    Stephanie Griemsmann
    Institute of Cellular Neurosciences, Medical Faculty, University of Bonn, 53105 Bonn, Germany
    Cereb Cortex 25:3420-33. 2015
    ..Cxs), semiquantitative reverse transcription-polymerase chain reaction (RT-PCR) and western blotting showed that Cx30 is the dominant astrocytic Cx in the thalamus...
  30. ncbi Expression of zonula occludens-1 (ZO-1) and the transcription factor ZO-1-associated nucleic acid-binding protein (ZONAB)-MsY3 in glial cells and colocalization at oligodendrocyte and astrocyte gap junctions in mouse brain
    Mihai C Penes
    Department of Physiology, Faculty of Medicine, University of Manitoba, 730 William Ave, Winnipeg, Manitoba R3E 3J7, Canada
    Eur J Neurosci 22:404-18. 2005
    ..with astrocytic connexins by confocal immunofluorescence demonstration of ZO-1 colocalization with astrocytic Cx30 and Cx43, and by ZO-1 coimmunoprecipitation with Cx30 and Cx43...
  31. pmc Assembly of the cochlear gap junction macromolecular complex requires connexin 26
    Kazusaku Kamiya
    J Clin Invest 124:1598-607. 2014
    ..Surprisingly, CX26 deficiency is not compensated for by the closely related connexin CX30, which is abundantly expressed in the same cochlear cells...
  32. pmc Deafness induced by Connexin 26 (GJB2) deficiency is not determined by endocochlear potential (EP) reduction but is associated with cochlear developmental disorders
    Jin Chen
    Dept of Otolaryngology, University of Kentucky Medical School, Lexington, KY 40536, USA Department of Otolaryngology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, PR China
    Biochem Biophys Res Commun 448:28-32. 2014
    ..These data suggest that congenital deafness induced by Cx26 deficiency is not determined by EP reduction and may result from cochlear developmental disorders...
  33. doi Connexin 30 sets synaptic strength by controlling astroglial synapse invasion
    Ulrike Pannasch
    1 Center for Interdisciplinary Research in Biology, College de France, INSERM U1050, CNRS UMR 7241, Labex Memolife, PSL Research University, Paris, France 2 Doctoral school N 158, Pierre and Marie Curie University, Paris, France 3
    Nat Neurosci 17:549-58. 2014
    Astrocytes play active roles in brain physiology by dynamic interactions with neurons. Connexin 30, one of the two main astroglial gap-junction subunits, is thought to be involved in behavioral and basic cognitive processes...
  34. pmc Fast structural responses of gap junction membrane domains to AB5 toxins
    Irina V Majoul
    Institute of Biology, Center for Structural and Cell Biology in Medicine, University of Lubeck, 23562 Lübeck, Germany
    Proc Natl Acad Sci U S A 110:E4125-33. 2013
    ..Our data demonstrate very fast dynamics (in the millisecond-to-second range) within GJ plaques, which previously were considered to be relatively stable, long-lived structures...
  35. doi Ablation of connexin30 in transgenic mice alters expression patterns of connexin26 and connexin32 in glial cells and leptomeninges
    B D Lynn
    Department of Physiology, Faculty of Medicine, University of Manitoba, Winnipeg, Manitoba, Canada
    Eur J Neurosci 34:1783-93. 2011
    Expression of connexin26 (Cx26), Cx30 and Cx43 in astrocytes and expression of Cx29, Cx32 and Cx47 in oligodendrocytes of adult rodent brain has been well documented, as has the interdependence of connexin expression patterns of ..
  36. pmc Early developmental expression of connexin26 in the cochlea contributes to its dominate functional role in the cochlear gap junctions
    Yan Qu
    Department of Ear Nose and Throat, Third Hospital and Bethune International Peace Hospital of Hebei Medical University, Shijiazhuang, Hebei, People s Republic of China
    Biochem Biophys Res Commun 417:245-50. 2012
    Mutations in Gjb2 and Gjb6 genes, coding for connexin26 (Cx26) and Cx30 proteins, respectively, are linked to about half of all cases of human autosomal non-syndromic prelingual deafness...
  37. doi Loss of astrocyte connexins 43 and 30 does not significantly alter susceptibility or severity of acute experimental autoimmune encephalomyelitis in mice
    Sarah E Lutz
    Department of Neuroscience, Albert Einstein College of Medicine, Bronx, NY, USA
    J Neuroimmunol 245:8-14. 2012
    We showed previously that mice deficient in astrocyte gap junctions Cx43 and Cx30 exhibit white matter vacuolation and hypomyelination...
  38. pmc Critical role of connexin 43 in secondary expansion of traumatic spinal cord injury
    Chunlan Huang
    Division of Glial Disease and Therapeutics, Center for Translational Neuromedicine, Department of Neurosurgery, University of Rochester, Rochester, New York 14642, USA
    J Neurosci 32:3333-8. 2012
    ..for astrocytic ATP release, we here evaluated the impact on post-traumatic ATP release of deletion of connexins (Cx30/Cx43) in astrocytes...
  39. pmc Purinergic inhibition of ENaC produces aldosterone escape
    James D Stockand
    Department of Physiology, University of Texas Health Science Center at San Antonio, San Antonio, TX 78229 3900, USA
    J Am Soc Nephrol 21:1903-11. 2010
    ..These data suggest that control of ENaC by purinergic signaling is necessary for aldosterone escape...
  40. pmc Deletion of astroglial connexins weakens the blood-brain barrier
    Pascal Ezan
    Center for Interdisciplinary Research in Biology Centre National de la Recherche Scientifique, College de France, Unité Mixte de Recherche 7241 Institut National de la Santé et de la Recherche Médicale U1050, Paris, France
    J Cereb Blood Flow Metab 32:1457-67. 2012
    ..striking properties of astrocyte endfeet is their enrichment in gap junction protein connexins 43 and 30 (Cx43 and Cx30) allowing for direct intercellular trafficking of ions and small signaling molecules through perivascular ..
  41. doi Panglial gap junctional communication is essential for maintenance of myelin in the CNS
    Oliver Tress
    Institute of Genetics, Division of Molecular Genetics, University of Bonn, 53117 Bonn, Germany
    J Neurosci 32:7499-518. 2012
    In this study, we have investigated the contribution of oligodendrocytic connexin47 (Cx47) and astrocytic Cx30 to panglial gap junctional networks as well as myelin maintenance and function by deletion of both connexin coding DNAs in mice...
  42. doi Dual reporter approaches for identification of Cre efficacy and astrocyte heterogeneity
    Joachim Degen
    Life and Medical Sciences Institute, Medical Faculty, University of Bonn, Bonn, Germany
    FASEB J 26:4576-83. 2012
    ..Analysis of a newly generated Cx43 knock-in ECFP mouse revealed an unexpected heterogeneity of Cx43-expressing astrocytes across brain areas...
  43. pmc Reduced phosphatidylinositol 4,5-bisphosphate synthesis impairs inner ear Ca2+ signaling and high-frequency hearing acquisition
    Laura Rodriguez
    Istituto Veneto di Medicina Molecolare, Fondazione per la Ricerca Biomedica Avanzata, 35129 Padua, Italy
    Proc Natl Acad Sci U S A 109:14013-8. 2012
    ..This mouse model also suggests that PIPKIγ may determine the level of gap junction contribution to cochlear development...
  44. pmc Astrocytic CX43 hemichannels and gap junctions play a crucial role in development of chronic neuropathic pain following spinal cord injury
    Michael J Chen
    Department of Neurosurgery, Center for Translational Neuromedicine, University of Rochester Medical School, Rochester, New York, USA
    Glia 60:1660-70. 2012
    ..the extent of Cx43 involvement in chronic pain, a weight-drop SCI was performed on transgenic mice with Cx43/Cx30 deletions...
  45. doi GJB6, of which mutations underlie Clouston syndrome, is a potential direct target gene of p63
    Atsushi Fujimoto
    Laboratory of Genetic Skin Diseases, Niigata University Graduate School of Medical and Dental Sciences, Niigata, Japan
    J Dermatol Sci 69:159-66. 2013
    ..The disease is caused by mutations in GJB6 gene, which encodes a gap junction protein connexin 30 (Cx30).
  46. doi Connexin47 protein phosphorylation and stability in oligodendrocytes depend on expression of Connexin43 protein in astrocytes
    Dennis May
    Life and Medical Sciences Institute, Molecular Genetics, University of Bonn, D 53115 Bonn, Germany
    J Neurosci 33:7985-96. 2013
    ..These results further unravel the complexity of panglial networks and show that results of previous studies using astrocytic Cx43-deficient mice have to be reconsidered...
  47. doi Hearing is normal without connexin30
    Anne Cécile Boulay
    College de France, Centre Interdisciplinaire de Recherche en Biologie CIRB Centre National de la Recherche Scientifique CNRS, Unité Mixte de Recherche UMR 7241 Institut National de la Santé et de la Recherche Médicale Inserm, U1050, 75231 Paris Cedex 05, France
    J Neurosci 33:430-4. 2013
    Gjb2 and Gjb6, two contiguous genes respectively encoding the gap junction protein connexin26 (Cx26) and connexin 30 (Cx30) display overlapping expression in the inner ear...
  48. pmc A pair of co-opted retroviral envelope syncytin genes is required for formation of the two-layered murine placental syncytiotrophoblast
    Anne Dupressoir
    Unité des rétrovirus endogènes et éléments rétroïdes des eucaryotes supérieurs, Centre National de la Recherche Scientifique, Unité Mixte de Recherche 8122, and Unité de Génomique Fonctionnelle, Institut Gustave Roussy, 94805 Villejuif, France
    Proc Natl Acad Sci U S A 108:E1164-73. 2011
    ..Microarray analyses identified up-regulation of the connexin 30 gene in mutant placentae, with the protein localized at the fetomaternal interface, suggesting gap junction-..
  49. pmc SMAD 8 binding to mice Msx1 basal promoter is required for transcriptional activation
    Renata Binato
    Laboratorio de Biologia Molecular Maury Miranda, Instituto de Biofisica Carlos Chagas Filho, Universidade Federal do Rio de Janeiro, Rio de Janeiro, RJ, Brazil
    Biochem J 393:141-50. 2006
    ..Our results suggest that BMP2/BMP4 signalling through SMAD 8 is required for transcriptional activation of the mouse Msx1 gene...
  50. doi Milk secretion and ejection are impaired in the mammary gland of mice harboring a Cx43 mutant while expression and localization of tight and adherens junction proteins remain unchanged
    Isabelle Plante
    Department of Anatomy and Cell Biology, University of Western Ontario, London, Ontario, Canada
    Biol Reprod 82:837-47. 2010
    ..Surprisingly, the levels and/or localization of Cx26 (Gjb2), Cx30 (Gjb6), Cx32 (Gjb1), E-cadherin, P-cadherin, beta-catenin, claudin-1, and occludin were all unaffected by the ..
  51. doi Connexin-deficiency affects expression levels of glial glutamate transporters within the cerebrum
    Tina Unger
    Institute of Anatomy, University of Leipzig, Medical Faculty, Liebigstr 13, 04103 Leipzig, Germany
    Neurosci Lett 506:12-6. 2012
    ..transporters, we have now assessed their expression levels in brains of conditional Cx43 knockout mice, total Cx30 knockouts, as well as Cx43/Cx30 double knockouts...
  52. pmc Plasticity of astroglial networks in olfactory glomeruli
    Lisa Roux
    College de France, Centre National de la Recherche Scientifique, Unité Mixte de Recherche 7241 Institut National de la Santé et de la Recherche Médicale U1050, 75231 Paris Cedex 05, France
    Proc Natl Acad Sci U S A 108:18442-6. 2011
    ..of glomerular astrocytes to the glomerulus center and the enriched expression of two astroglial connexins (Cx43 and Cx30) within the glomeruli...
  53. doi Gap junctions mediate intercellular spread of sodium between hippocampal astrocytes in situ
    Julia Langer
    Institute of Neurobiology, Heinrich Heine University Duesseldorf, Universitatsstrasse 1, Duesseldorf, Germany
    Glia 60:239-52. 2012
    ..was disturbed on pharmacological inhibition of gap junctions, reduced in animals at P4 and virtually omitted in Cx30/Cx43 double-deficient mice...
  54. ncbi Human connexin 30 (GJB6), a candidate gene for nonsyndromic hearing loss: molecular cloning, tissue-specific expression, and assignment to chromosome 13q12
    P M Kelley
    Center for Hereditary Communication Disorders, Boys Town National Research Hospital, Omaha, Nebraska 68131, USA
    Genomics 62:172-6. 1999
    ..Another gap junction gene, connexin 30 (HGMW-approved symbol GJB6), is found to lie on the same PAC clone that hybridizes to chromosome 13q12...
  55. ncbi Functional significance of gap junctional coupling in preimplantation development
    F D Houghton
    Department of Physiology, Dental Sciences Building, The University of Western Ontario, London, Ontario, Canada N6A 5C1
    Biol Reprod 66:1403-12. 2002
    ..from reverse transcription-polymerase chain reaction analysis that the genes encoding 3 additional connexins (connexin 30 or beta6, connexin 36 or alpha9, and connexin 57 or alpha10) are also transcribed in preimplantation mouse ..
  56. ncbi Altered connexin expression and wound healing in the epidermis of connexin-deficient mice
    Markus Kretz
    Institut fur Genetik, Abteilung Molekulargenetik Römerstrasse 164, 53117 Bonn, Germany
    J Cell Sci 116:3443-52. 2003
    ..These results suggest that downregulation of connexin43 appears to be a prerequisite for the coordinated proliferation and mobilization of keratinocytes during wound healing...
  57. ncbi Connexin30-deficient mice show increased emotionality and decreased rearing activity in the open-field along with neurochemical changes
    E Dere
    Institute of Physiological Psychology, Center for Biological and Medical Research, Heinrich Heine University of Dusseldorf, Germany
    Eur J Neurosci 18:629-38. 2003
    ..Thus, we have tested groups of Cx30-/-, Cx30+/-, and Cx30+/+ mice in the open-field, an object exploration task, in the graded anxiety test and on the ..
  58. ncbi An update on connexin genes and their nomenclature in mouse and man
    Goran Sohl
    Institut fur Genetik, Universitat Bonn, Germany
    Cell Commun Adhes 10:173-80. 2003
    ..Furthermore, this short review contains an update on phenotypic correlations between connexin deficient mice and patients bearing mutations in their orthologous connexin genes...
  59. ncbi An expression atlas of connexin genes in the mouse
    Annalisa Buniello
    Telethon Institute of Genetics and Medicine, Via P Castellino 111, 80131 Naples, Italy
    Genomics 83:812-20. 2004
    ....
  60. ncbi The novel mouse connexin39 gene is expressed in developing striated muscle fibers
    Julia von Maltzahn
    Institut fur Genetik, Abteilung Molekulargenetik, Universitat Bonn, Römerstr 164, 53117 Bonn, Germany
    J Cell Sci 117:5381-92. 2004
    ..As expression of no other known connexin could be verified in these cells, intercellular dye transfer might result from functional expression of Cx39 in developing striated muscle fibers...
  61. ncbi Connexin30 in rodent, cat and human brain: selective expression in gray matter astrocytes, co-localization with connexin43 at gap junctions and late developmental appearance
    J I Nagy
    Department of Physiology, Faculty of Medicine, University of Manitoba, Winnipeg, Canada
    Neuroscience 88:447-68. 1999
    ..Taken together, these results suggest that astrocytic connexin30 expression at both regional and cellular levels is subject to regulation in adult brain as well as during brain development...
  62. pmc Strain background effects and genetic modifiers of hearing in mice
    Kenneth R Johnson
    The Jackson Laboratory, Bar Harbor, ME 04609, USA
    Brain Res 1091:79-88. 2006
    ..Because modifier genes and digenic inheritance are not always distinguishable, we also include in this review several examples of digenic inheritance of hearing loss that have been reported in both mice and humans...
  63. ncbi Nature of Cx30-containing channels in the adult mouse mammary gland
    Darren Locke
    Department of Pharmacology and Physiology, New Jersey Medical School, UMDNJ, Newark, NJ 07103, USA
    Cell Tissue Res 328:97-107. 2007
    ..Additionally, Cx30 is abundant in late-pregnant and early lactating gland epithelium...
  64. ncbi The connexin31 F137L mutant mouse as a model for the human skin disease erythrokeratodermia variabilis (EKV)
    Marc Schnichels
    Institut fur Genetik, Abteilung Molekulargenetik, Universitat Bonn, Bonn 53117, Germany
    Hum Mol Genet 16:1216-24. 2007
    ..So far, several mutations in the Cx31 or Cx30.3 gene have been reported to cause EKV in humans...
  65. ncbi Expression of connexins in embryonic mouse neocortical development
    Cima Cina
    Department of Cellular and Physiological Sciences, University of British Columbia, Vancouver, British Columbia, V6T 1Z3, Canada
    J Comp Neurol 504:298-313. 2007
    ..family of gap junction proteins, Cx26, Cx36, Cx37, Cx43, and Cx45 were expressed in the developing cortex of mice, Cx30 and Cx32 were absent, while Cx40 was expressed at a very low level...
  66. pmc Analysis of connexin subunits required for the survival of vestibular hair cells
    Yan Qu
    Department of Otolaryngology, Emory University School of Medicine, Atlanta, Georgia 30322 3030, USA
    J Comp Neurol 504:499-507. 2007
    ..The most commonly found human Cx mutations are either Cx26 or Cx30 deletions...
  67. doi A central role of connexin 43 in hypoxic preconditioning
    Jane H C Lin
    Department of Pathology, New York Medical College, Valhalla, New York 10595, USA
    J Neurosci 28:681-95. 2008
    ..Thus, adaptive modulation of Cx43 can offset environmental stress by adenosine-mediated elevation of cellular resistance...
  68. doi Neurons control the expression of connexin 30 and connexin 43 in mouse cortical astrocytes
    Annette Koulakoff
    INSERM, U840, College de France, 11 place Marcelin Berthelot, Paris, France
    Glia 56:1299-311. 2008
    ..The two main connexins, Cx30 and Cx43, that form these junctions in astrocytes of adult brain display different developmental and regional ..
  69. pmc Gap junction mediated intercellular metabolite transfer in the cochlea is compromised in connexin30 null mice
    Qing Chang
    Department of Otolaryngology, Emory University School of Medicine, Atlanta, GA, USA
    PLoS ONE 3:e4088. 2008
    Connexin26 (Cx26) and connexin30 (Cx30) are two major protein subunits that co-assemble to form gap junctions (GJs) in the cochlea. Mutations in either one of them are the major cause of non-syndromic prelingual deafness in humans...
  70. pmc Connexin 30 deficiency impairs renal tubular ATP release and pressure natriuresis
    Arnold Sipos
    Department of Physiology and Biophysics and Department of Medicine, Zilkha Neurogenetic Institute, University of Southern California, Los Angeles, CA 90033, USA
    J Am Soc Nephrol 20:1724-32. 2009
    ..Several connexin (Cx) isoforms form mechanosensitive, ATP-permeable hemichannels. We localized Cx30 to the nonjunctional apical membrane of cells in the distal nephron and tested whether Cx30 participates in ..
  71. pmc Deletion of astrocyte connexins 43 and 30 leads to a dysmyelinating phenotype and hippocampal CA1 vacuolation
    Sarah E Lutz
    Department of Pathology, Albert Einstein College of Medicine, Bronx, New York 10461, USA
    J Neurosci 29:7743-52. 2009
    Astrocytes are coupled via gap junctions (GJs) comprising connexin 43 (Cx43) (Gja1) and Cx30 (Gjb6), which facilitate intercellular exchange of ions. Astrocyte connexins also form heterotypic GJs with oligodendrocytic somata and lamellae...
  72. doi Connexin 30 is expressed in the mouse sino-atrial node and modulates heart rate
    Daniel Gros
    Institut de Biologie du Developpement de Marseille Luminy, Universite de la Mediterranee, Marseille, France
    Cardiovasc Res 85:45-55. 2010
    This study aimed at characterizing expression and the functional role of the Gjb6 gene, encoding for connexin 30 (Cx30) protein, in the adult mouse heart.
  73. pmc Consortin, a trans-Golgi network cargo receptor for the plasma membrane targeting and recycling of connexins
    Francisco J del Castillo
    Unite de Genetique et Physiologie de l Audition, Institut Pasteur, Paris, France
    Hum Mol Genet 19:262-75. 2010
    ..Sequence accession numbers. GenBank: Human CNST cDNA, NM_152609; mouse Cnst cDNA, NM_146105...
  74. ncbi Molecular cloning and functional expression of mouse connexin-30,a gap junction gene highly expressed in adult brain and skin
    E Dahl
    Institut fur Genetik, Abteilung Molekulargenetik, Universitat Bonn, 53117 Bonn, Federal Republic of Germany
    J Biol Chem 271:17903-10. 1996
    ..This suggests that the polarity of voltage gating and the cationic selectivity of connexin-30 are similar to those of its closest homologue, connexin-26...
  75. pmc The human deafness-associated connexin 30 T5M mutation causes mild hearing loss and reduces biochemical coupling among cochlear non-sensory cells in knock-in mice
    Melanie Schütz
    Institut fuer Genetik, Rheinische Friedrich Wilhelms Universitaet Bonn, Roemerstrasse 164, D 53117 Bonn, Germany
    Hum Mol Genet 19:4759-73. 2010
    Mutations in the GJB2 and GJB6 genes, respectively, coding for connexin26 (Cx26) and connexin30 (Cx30) proteins, are the most common cause for prelingual non-syndromic deafness in humans...
  76. pmc Diminished paracrine regulation of the epithelial Na+ channel by purinergic signaling in mice lacking connexin 30
    Elena Mironova
    Department of Physiology, University of Texas Health Science Center, San Antonia, Texas 78229 3900, USA
    J Biol Chem 286:1054-60. 2011
    We tested whether ATP release through Connexin 30 (Cx30) is part of a local purinergic regulatory system intrinsic to the aldosterone-sensitive distal nephron (ASDN) important for proper control of sodium excretion; if changes in sodium ..
  77. doi Dysfunction of astrocyte connexins 30 and 43 in dorsal lateral prefrontal cortex of suicide completers
    Carl Ernst
    McGill Group for Suicide Studies, McGill University, Montreal, Quebec, Canada
    Biol Psychiatry 70:312-9. 2011
    ..Although it is known that particular neurobiological processes underlie suicidal ideation and behavior, there still remains limited knowledge about the specific factors involved...

Research Grants2

  1. Epithelial Na channel (ENaC) polymorphisms in hyptertention
    James Stockand; Fiscal Year: 2007
    ..Preliminary results support the feasibility of this investigation and suggest that this line of inquiry will yield significant and novel findings. [unreadable] [unreadable]..
  2. Stoichiometry and modular retrieval of ENaC
    James Stockand; Fiscal Year: 2009
    ..This research will provide critical insight about the molecualr architecture of ENaC and regulation of this important channel. ..