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Human oligodendrocytes express Cx31.3: function and interactions with Cx32 mutants.

Abstract
Murine oligodendrocytes express the gap junction (GJ) proteins connexin32 (Cx32), Cx47, and Cx29. CNS phenotypes in patients with X-linked Charcot-Marie-Tooth disease may be caused by dominant effects of Cx32 mutations on other connexins. Here we examined the expression of Cx31.3 (the human ortholog of murine Cx29) in human brain and its relation to the other oligodendrocyte GJ proteins Cx32 and Cx47. Furthermore, we investigated in vitro whether Cx32 mutants with CNS manifestations affect the expression and function of Cx31.3. Cx31.3 was localized mostly in the gray matter along small myelinated fibers similar to Cx29 in rodent brain and was co-expressed with Cx32 in a subset of human oligodendrocytes. In HeLa cells Cx31.3 was localized at the cell membrane and appeared to form hemichannels but no GJs. Cx32 mutants with CNS manifestations were retained intracellularly, but did not alter the cellular localization or function of co-expressed Cx31.3. Thus, Cx31.3 shares many characteristics with its ortholog Cx29. Cx32 mutants with CNS phenotypes do not affect the trafficking or function of Cx31.3, and may have other toxic effects in oligodendrocytes.
AuthorsIrene Sargiannidou, Meejin Ahn, Alan D Enriquez, Alejandro Peinado, Richard Reynolds, Charles Abrams, Steven S Scherer, Kleopas A Kleopa
JournalNeurobiology of disease (Neurobiol Dis) Vol. 30 Issue 2 Pg. 221-33 (May 2008) ISSN: 1095-953X [Electronic] United States
PMID18353664 (Publication Type: Comparative Study, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
Chemical References
  • Connexins
  • GJC3 protein, human
  • Nerve Tissue Proteins
Topics
  • Amino Acid Sequence
  • Cell Communication (genetics)
  • Connexins (biosynthesis, genetics, physiology)
  • Gene Expression Regulation (physiology)
  • HeLa Cells
  • Humans
  • Molecular Sequence Data
  • Mutation
  • Nerve Tissue Proteins (biosynthesis, genetics, physiology)
  • Oligodendroglia (chemistry, physiology)
  • Gap Junction beta-1 Protein

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