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

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

SUBMITTER: Chrast R 

PROVIDER: S-EPMC3035679 | biostudies-literature | 2011 Mar

REPOSITORIES: biostudies-literature