Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
1996-9-6
pubmed:abstractText
The vertebrate nervous system is characterized by ensheathment of axons with myelin, a multilamellar membrane greatly enriched in the galactolipid galactocerebroside (GalC) and its sulfated derivative sulfatide. We have generated mice lacking the enzyme UDP-galactose:ceramide galactosyltransferase (CGT), which is required for GalC synthesis. CGT-deficient mice do not synthesize GalC or sulfatide but surprisingly form myelin containing glucocerebroside, a lipid not previously identified in myelin. Microscopic and morphometric analyses revealed myelin of normal ultrastructural appearance, except for slightly thinner sheaths in the ventral region of the spinal cord. Nevertheless, these mice exhibit severe generalized tremoring and mild ataxia, and electrophysiological analysis showed conduction deficits consistent with reduced insulative capacity of the myelin sheath. Moreover, with age, CGT-deficient mice develop progressive hindlimb paralysis and extensive vacuolation of the ventral region of the spinal cord. These results indicate that GalC and sulfatide play important roles in myelin function and stability.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jul
pubmed:issn
0092-8674
pubmed:author
pubmed:issnType
Print
pubmed:day
26
pubmed:volume
86
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
209-19
pubmed:dateRevised
2007-11-14
pubmed:meshHeading
pubmed-meshheading:8706126-Animals, pubmed-meshheading:8706126-Brain Chemistry, pubmed-meshheading:8706126-Electrophysiology, pubmed-meshheading:8706126-Fatty Acids, pubmed-meshheading:8706126-Female, pubmed-meshheading:8706126-Galactosylceramides, pubmed-meshheading:8706126-Galactosyltransferases, pubmed-meshheading:8706126-Lipid Metabolism, pubmed-meshheading:8706126-Mice, pubmed-meshheading:8706126-Mice, Knockout, pubmed-meshheading:8706126-Microscopy, Electron, pubmed-meshheading:8706126-Mutagenesis, pubmed-meshheading:8706126-Myelin Sheath, pubmed-meshheading:8706126-Nerve Fibers, Myelinated, pubmed-meshheading:8706126-Neural Conduction, pubmed-meshheading:8706126-Optic Nerve, pubmed-meshheading:8706126-Phenotype, pubmed-meshheading:8706126-Sciatic Nerve, pubmed-meshheading:8706126-Sulfoglycosphingolipids
pubmed:year
1996
pubmed:articleTitle
Myelination in the absence of galactocerebroside and sulfatide: normal structure with abnormal function and regional instability.
pubmed:affiliation
UNC Neuroscience Center, University of North Carolina at Chapel Hill 27599, USA.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S., Research Support, Non-U.S. Gov't