Connexins are critical for normal myelination in the CNS

Daniela M. Menichella, Daniel A. Goodenough, Erich Sirkowski, Steven S. Scherer, David L. Paul*

*Corresponding author for this work

Research output: Contribution to journalArticle

221 Scopus citations

Abstract

Mutations in Cx32, a gap-junction channel-forming protein, result in X-linked Charcot-Marie-Tooth disease, a demyelinating disease of the peripheral nervous system. However, although oligodendrocytes express Cx32, central myelination is unaffected. To explore this discrepancy, we searched for additional oligodendrocyte connexins. We found Cx47, which is expressed specifically in oligodendrocytes, regulated in parallel with myelin genes and partially colocalized with Cx32 in oligodendrocytes. Mice lacking either Cx47 or Cx32 are viable. However, animals lacking both connexins die by postnatal week 6 from profound abnormalities in central myelin, characterized by thin or absent myelin sheaths, vacuolation, enlarged periaxonal collars, oligodendrocyte cell death, and axonal loss. These data provide the first evidence that gap-junction communication is crucial for normal central myelination.

Original languageEnglish (US)
Pages (from-to)5963-5973
Number of pages11
JournalJournal of Neuroscience
Volume23
Issue number13
DOIs
StatePublished - Jul 1 2003

Keywords

  • Connexin
  • Cx32
  • Cx47
  • Gap junction
  • Myelin
  • Oligodendrocyte

ASJC Scopus subject areas

  • Neuroscience(all)

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