RT Journal Article
SR Electronic
T1 Connexins Are Critical for Normal Myelination in the CNS
JF The Journal of Neuroscience
JO J. Neurosci.
FD Society for Neuroscience
SP 5963
OP 5973
DO 10.1523/JNEUROSCI.23-13-05963.2003
VO 23
IS 13
A1 Daniela M. Menichella
A1 Daniel A. Goodenough
A1 Erich Sirkowski
A1 Steven S. Scherer
A1 David L. Paul
YR 2003
UL http://www.jneurosci.org/content/23/13/5963.abstract
AB 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.