RT Journal Article
SR Electronic
T1 Myelin Galactolipids Are Essential for Proper Node of Ranvier Formation in the CNS
JF The Journal of Neuroscience
JO J. Neurosci.
FD Society for Neuroscience
SP 1642
OP 1649
DO 10.1523/JNEUROSCI.18-05-01642.1998
VO 18
IS 5
A1 Jeffrey L. Dupree
A1 Timothy Coetzee
A1 Andrew Blight
A1 Kinuko Suzuki
A1 Brian Popko
YR 1998
UL http://www.jneurosci.org/content/18/5/1642.abstract
AB The vertebrate myelin sheath is greatly enriched in the galactolipids galactocerebroside (GalC) and sulfatide. Mice with a disruption in the gene that encodes the biosynthetic enzyme UDP-galactose:ceramide galactosyl transferase (CGT) are incapable of synthesizing these lipids yet form myelin sheaths that exhibit major and minor dense lines with spacing comparable to controls. These CGT mutant mice exhibit a severe tremor that is accompanied by hindlimb paralysis. Furthermore, electrophysiological studies reveal nerve conduction deficits in the spinal cord of these mutants. Here, using electron microscopic techniques, we demonstrate ultrastructural myelin abnormalities in the CNS that are consistent with the electrophysiological deficits. These abnormalities include altered nodal lengths, an abundance of heminodes, an absence of transverse bands, and the presence of reversed lateral loops. In contrast to the CNS, no ultrastructural abnormalities and only modest electrophysiological deficits were observed in the peripheral nervous system. Taken together, the data presented here indicate that GalC and sulfatide are essential in proper CNS node and paranode formation and that these lipids are important in ensuring proper axo-oligodendrocyte interactions.