Lipid metabolism in myelinating glial cells: lessons from human inherited disorders and mouse models.

Details

Serval ID
serval:BIB_8903A86C970E
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
Lipid metabolism in myelinating glial cells: lessons from human inherited disorders and mouse models.
Journal
Journal of Lipid Research
Author(s)
Chrast R., Saher G., Nave K.A., Verheijen M.H.
ISSN
0022-2275 (Print)
ISSN-L
0022-2275
Publication state
Published
Issued date
2011
Volume
52
Number
3
Pages
419-434
Language
english
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't
Abstract
The integrity of central and peripheral nervous system myelin is affected in numerous lipid metabolism disorders. This vulnerability was so far mostly attributed to the extraordinarily high level of lipid synthesis that is required for the formation of myelin, and to the relative autonomy in lipid synthesis of myelinating glial cells because of blood barriers shielding the nervous system from circulating lipids. Recent insights from analysis of inherited lipid disorders, especially those with prevailing lipid depletion and from mouse models with glia-specific disruption of lipid metabolism, shed new light on this issue. The particular lipid composition of myelin, the transport of lipid-associated myelin proteins, and the necessity for timely assembly of the myelin sheath all contribute to the observed vulnerability of myelin to perturbed lipid metabolism. Furthermore, the uptake of external lipids may also play a role in the formation of myelin membranes. In addition to an improved understanding of basic myelin biology, these data provide a foundation for future therapeutic interventions aiming at preserving glial cell integrity in metabolic disorders.
Pubmed
Web of science
Open Access
Yes
Create date
08/03/2011 9:39
Last modification date
20/08/2019 14:48
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