Document Detail

Myelination in the absence of galactolipids and proteolipid proteins.
MedLine Citation:
PMID:  10433816     Owner:  NLM     Status:  MEDLINE    
The galactolipids galactocerebroside and sulfatide and the proteolipid protein (PLP) and its splice variant DM20 are the most abundant lipid and protein components of central nervous system myelin. Recent studies have found that mice lacking either the galactolipids or PLP are able to form myelin sheaths with apparently normal periodicity and near normal compaction. Here, we have generated galactolipid/proteolipid double mutants to examine the possibility that these molecules have overlapping functions. We show that the absence of the galactolipids and PLP has pleotropic effects on myelin formation. While oligodendrocytes in the postnatal day 20 galactolipid/proteolipid-deficient mouse are able to elaborate myelin with close to normal intraperiod lines, there is an increased frequency of uncompacted myelin sheaths as well as unmyelinated axons. Moreover, the double mutants display extensive white matter vacuolization of the cerebellum that initiates around postnatal day 16, which correlates with the onset of a severe ataxic phenotype and an increased percentage of apoptotic nuclei in the cerebellar internal granule cell layer. These data indicate that the galactolipids and PLP/DM20 are not required for intraperiod line formation, but they suggest a role for these molecules in mediating myelin compaction and in maintaining the integrity of the cerebellum.
T Coetzee; K Suzuki; K A Nave; B Popko
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Molecular and cellular neurosciences     Volume:  14     ISSN:  1044-7431     ISO Abbreviation:  Mol. Cell. Neurosci.     Publication Date:  1999 Jul 
Date Detail:
Created Date:  1999-09-24     Completed Date:  1999-09-24     Revised Date:  2008-11-21    
Medline Journal Info:
Nlm Unique ID:  9100095     Medline TA:  Mol Cell Neurosci     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  41-51     Citation Subset:  IM    
Copyright Information:
Copyright 1999 Academic Press.
Department of Pathology and Laboratory Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina 27599, USA.
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MeSH Terms
Alternative Splicing
Brain / pathology,  physiology*,  ultrastructure
Cerebellum / pathology
Crosses, Genetic
Genetic Variation
Glycolipids / deficiency,  physiology*
Mice, Knockout
Myelin Proteolipid Protein / deficiency,  genetics,  physiology*
Myelin Sheath / physiology*,  ultrastructure
Nerve Degeneration / genetics*,  pathology
Polymerase Chain Reaction
Grant Support
Reg. No./Substance:
0/Galactolipids; 0/Glycolipids; 0/Myelin Proteolipid Protein

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