Cholesterol in myelin biogenesis and hypomyelinating disorders
- Resource Type
- Authors
- Sina K. Stumpf; Gesine Saher
- Source
- Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids. 1851:1083-1094
- Subject
- Central Nervous System
Pelizaeus-Merzbacher Disease
Central nervous system
Biology
Synaptic Transmission
Myelin
Peripheral Nervous System
medicine
Animals
Homeostasis
Humans
Molecular Biology
Myelin Sheath
Neurons
Niemann–Pick disease, type C
Cell Membrane
Neurodegeneration
Pelizaeus–Merzbacher disease
Niemann-Pick Disease, Type C
Cell Biology
medicine.disease
Oligodendrocyte
Smith-Lemli-Opitz Syndrome
Cell biology
Cholesterol
medicine.anatomical_structure
nervous system
Biochemistry
Smith–Lemli–Opitz syndrome
Astrocytes
lipids (amino acids, peptides, and proteins)
Biogenesis
- Language
- ISSN
- 1388-1981
The largest pool of free cholesterol in mammals resides in myelin membranes. Myelin facilitates rapid saltatory 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.