Journal
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS
Volume 1851, Issue 8, Pages 1083-1094Publisher
ELSEVIER
DOI: 10.1016/j.bbalip.2015.02.010
Keywords
Myelin; Cholesterol; Pelizaeus-Merzbacher disease; Smith-Lemli-Opitz syndrome; Niemann-Pick disease type C; Oligodendrocyte
Funding
- German Research Foundation [SA2114/1-1]
- European Leukodystrophy Association [2012-036I1]
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The largest pool of free cholesterol in mammals resides in myelin membranes. Myelin facilitates rapid saltatoly impulse propagation by electrical insulation of axons. This function is achieved by ensheathing axons with a tightly compacted stack of membranes. Cholesterol influences myelination at many steps, from the differentiation of myelinating glial cells, over the process of myelin membrane biogenesis, to the functionality of mature myelin. Cholesterol emerged as the only integral myelin component that is essential and rate-limiting for the development of myelin in the central and peripheral nervous system. Moreover, disorders that interfere with sterol synthesis or intracellular trafficking of cholesterol and other lipids cause hypomyelination and neurodegeneration. This review summarizes recent results on the roles of cholesterol in CNS myelin biogenesis in normal development and under different pathological conditions. This article is part of a Special Issue entitled Brain Lipids. (c) 2015 Elsevier B.V. All rights reserved.
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