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Connexins are critical for normal myelination in the CNS.


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.

SUBMITTER: Menichella DM 

PROVIDER: S-EPMC6741267 | biostudies-literature | 2003 Jul

REPOSITORIES: biostudies-literature

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Connexins are critical for normal myelination in the CNS.

Menichella Daniela M DM   Goodenough Daniel A DA   Sirkowski Erich E   Scherer Steven S SS   Paul David L DL  

The Journal of neuroscience : the official journal of the Society for Neuroscience 20030701 13


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. Mic  ...[more]

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