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

被引：235
作者：
Chrast, Roman [1 ]
Saher, Gesine [2 ]
Nave, Klaus-Armin [2 ]
Verheijen, Mark H. G. [3 ]
机构：
[1] Univ Lausanne, Dept Med Genet, CH-1015 Lausanne, Switzerland
[2] Max Planck Inst Expt Med, Dept Neurogenet, Gottingen, Germany
[3] Vrije Univ Amsterdam, Ctr Neurogenom & Cognit Res, Dept Mol & Cellular Neurobiol, Amsterdam, Netherlands
来源：
JOURNAL OF LIPID RESEARCH | 2011年 / 52卷 / 03期
基金：
瑞士国家科学基金会;
关键词：
LEMLI-OPITZ-SYNDROME; SJOGREN-LARSSON-SYNDROME; PERIPHERAL NERVOUS-SYSTEM; FATTY-ACID COMPOSITION; EXPERIMENTAL AUTOIMMUNE ENCEPHALOMYELITIS; PYRUVATE-CARBOXYLASE DEFICIENCY; GENOTYPE-PHENOTYPE CORRELATION; COA REDUCTASE INHIBITOR; LIVER PLASMA MEMBRANES; NIEMANN-PICK-DISEASE;
D O I：
10.1194/jlr.R009761
中图分类号：
Q5 [生物化学]; Q7 [分子生物学];
学科分类号：
071010 ; 081704 ;
摘要：
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. jlr 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.-Chrast, R., G. Saher, K-A. Nave, and M. H. G. Verheijen. Lipid metabolism in myelinating glial cells: lessons from human inherited disorders and mouse models. J. Lipid Res. 2011. 52: 419-434.
引用
收藏
页码：419 / 434
页数：16
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