title: Connexin 47 (Cx47)-deficient mice with enhanced green fluorescent protein reporter gene reveal predominant oligodendrocytic expression of Cx47 and display vacuolized myelin in the CNS.
abstract: To further characterize the recently described gap junction gene connexin 47 (Cx47), we generated Cx47-null mice by replacing the Cx47 coding DNA with an enhanced green fluorescent protein (EGFP) reporter gene, which was thus placed under control of the endogenous Cx47 promoter. Homozygous mutant mice were fertile and showed no obvious morphological or behavioral abnormalities. Colocalization of EGFP fluorescence and immunofluorescence of cell marker proteins revealed that Cx47 was mainly expressed in oligodendrocytes in highly myelinated CNS tissues and in few calcium-binding protein S100beta subunit-positive cells but not in neurons or peripheral sciatic nerve. This corrects our previous conclusion that Cx47 mRNA is expressed in brain and spinal cord neurons (Teubner et al., 2001). Cx47 protein was detected by Western blot analysis after immunoprecipitation in CNS tissues of wild-type mice but not in heart or Cx47-deficient tissues. Electron microscopic analysis of CNS white matter in Cx47-deficient mice revealed a conspicuous vacuolation of nerve fibers, particularly at the site of the optic nerve where axons are first contacted by oligodendrocytes and myelination starts. Initial analyses of Cx32/Cx47-double-deficient mice showed that these mice developed an action tremor and died on average at 51 d after birth. The central white matter of these double-deficient mice exhibited much more abundant vacuolation in nerve fibers than mice deficient only in Cx47.
authors.affiliation: Institut für Genetik, Universität Bonn, D-53117 Bonn, Germany.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Odermatt
authors.forename: Kerstin
authors.initials: K
authors.lastname: Wellershaus
authors.forename: Anke
authors.initials: A
authors.lastname: Wallraff
authors.forename: Gerald
authors.initials: G
authors.lastname: Seifert
authors.forename: Joachim
authors.initials: J
authors.lastname: Degen
authors.forename: Carsten
authors.initials: C
authors.lastname: Euwens
authors.forename: Babette
authors.initials: B
authors.lastname: Fuss
authors.forename: Heinrich
authors.initials: H
authors.lastname: Büssow
authors.forename: Karl
authors.initials: K
authors.lastname: Schilling
authors.forename: Christian
authors.initials: C
authors.lastname: Steinhäuser
authors.forename: Klaus
authors.initials: K
authors.lastname: Willecke
mesh_terms: D000818:Animals; D015153:Blotting, Western; D002490:Central Nervous System / Q000378:metabolism* / Q000473:pathology; D002999:Clone Cells; D017630:Connexins / Q000172:deficiency* / Q000235:genetics; D017629:Gap Junctions; D018390:Gene Targeting; D017930:Genes, Reporter; D049452:Green Fluorescent Proteins; D006367:HeLa Cells; D006720:Homozygote; D006801:Humans; D008164:Luminescent Proteins / Q000096:biosynthesis* / Q000235:genetics; D051379:Mice; D018345:Mice, Knockout; D008817:Mice, Mutant Strains; D009186:Myelin Sheath / Q000378:metabolism / Q000473:pathology*; D009836:Oligodendroglia / Q000378:metabolism* / Q000473:pathology; D009928:Organ Specificity; D018408:Patch-Clamp Techniques; D011401:Promoter Regions, Genetic; D012333:RNA, Messenger / Q000096:biosynthesis; D015996:Survival Rate; D014202:Tremor / Q000235:genetics / Q000473:pathology; D014617:Vacuoles / Q000473:pathology; D000097002:Gap Junction beta-1 Protein
chemical_list: D017630:Connexins; D049452:Green Fluorescent Proteins; D008164:Luminescent Proteins; D012333:RNA, Messenger; D000097002:Gap Junction beta-1 Protein; C427305:connexin 47