PT  - JOURNAL ARTICLE
AU  - Jeffrey L. Dupree
AU  - Timothy Coetzee
AU  - Andrew Blight
AU  - Kinuko Suzuki
AU  - Brian Popko
TI  - Myelin Galactolipids Are Essential for Proper Node of Ranvier Formation in the CNS
AID  - 10.1523/JNEUROSCI.18-05-01642.1998
DP  - 1998 Mar 01
TA  - The Journal of Neuroscience
PG  - 1642--1649
VI  - 18
IP  - 5
4099  - http://www.jneurosci.org/content/18/5/1642.short
4100  - http://www.jneurosci.org/content/18/5/1642.full
SO  - J. Neurosci.1998 Mar 01; 18
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.