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

Détails

ID Serval
serval:BIB_8903A86C970E
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Institution
Titre
Lipid metabolism in myelinating glial cells: lessons from human inherited disorders and mouse models.
Périodique
Journal of Lipid Research
Auteur(s)
Chrast R., Saher G., Nave K.A., Verheijen M.H.
ISSN
0022-2275 (Print)
ISSN-L
0022-2275
Statut éditorial
Publié
Date de publication
2011
Volume
52
Numéro
3
Pages
419-434
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't
Résumé
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
Oui
Création de la notice
08/03/2011 9:39
Dernière modification de la notice
20/08/2019 14:48
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