1970-08-01

glycosphingolipids; myelination; mouse mutant; maturation. ABSTRACT which are incapable of synthesizing the myelin galactolipids, here we show that there.

The further detailed analysis of these animals should help refine our understanding of galactolipid function in the myelination process. Among the most abundant components of myelin are the galactolipids galactocerebroside (GalC) and sulfatide. In spite of this abundance, the roles that these molecules play in the myelin sheath are not well understood. The vertebrate myelin sheath is greatly enriched in the galactolipids galactocerebroside (GalC) and sulfatide. Mice with a disruption in the gene that encodes the biosynthetic enzyme UDP-galactose:ceramide galactosyl transferase (CGT) are incapable of synthesizing these lipids yet form myelin sheaths that exhibit major and minor dense lines with spacing comparable to controls. The galactolipids galactocerebroside and sulfatide and the proteolipid protein (PLP) and its splice variant DM20 are the most abundant lipid and protein components of central nervous system myelin.

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These observations indicate that FA2H is a myelination-associated gene. FA2H-directed RNA interference (RNAi) by short-hairpin RNA expression resulted in a reduction of cellular 2-hydroxy fatty acids and 2-hydroxy GalCer in D6P2T Schwannoma cells, providing direct evidence that FA2H-dependent fatty acid 2-hydroxylation is required for the formation of 2-hydroxy galactolipids in peripheral Myelinated axons are divided into four distinct regions: the node of Ranvier, paranode, juxtaparanode, and internode, each of which is characterized by a specific set of axonal proteins. Voltage-gated Na+ channels are clustered at high densities at the nodes, whereas shaker-type K+ channels are concentrated at juxtaparanodal regions. These channels are separated by the paranodal regions, where The role of gangliosides in myelination is mainly associated with their ability to establish and modulate glia axon connections and MAG‐ganglioside downstream signalling (Schmitt et al., 2015). On the contrary of gangliosides, galactolipids, that is GalCer and sulphatides with long fatty acid chains (24:0 and 24:1), are abundant components of the myelin sheath.

The biochemical basis of the increase in 2-hydroxy galactolipids during myelination is now explained, at least in part, by the upregulation of FA2H in oligodendrocytes and Schwann cells.

(1999). Myelination in the absence of galactolipids and proteolipid proteins. (1994).

Myelination in the absence of galactolipids and proteolipid proteins. Coetzee T, Suzuki K, Nave KA, Popko B. Myelination in the absence of galactolipids and proteolipid proteins. Mol …

Despite the extraordinary abundance of 2-OH galactolipids in myelin, there is surprisingly little understanding of the basic biochemistry and physiological role of 2-OH galactolipids. The overall goal of this study is to elucidate the pathway for myelin 2-OH lipids and their roles in myelination, myelin function, and cell signaling. 2013-08-30 CiteSeerX - Document Details (Isaac Councill, Lee Giles, Pradeep Teregowda): We have analyzed mice that lack both the myelinassociated glycoprotein (MAG) and the myelin galactolipids, two glial components implicated in mediating axo-glial interactions during the myelination process. The single-mutant mice produce abnormal myelin containing similar ultrastructural abnormalities, suggesting that Epigenetic control of Myelination in PNS Review Review Molecular mechanisms regulating myelination in the peripheral nervous system Jorge A. Pereira, Fre´de´ric Lebrun-Julien and Ueli Suter Institute of Cell Biology, Department of Biology, Swiss Federal Institute of Technology, ETH Zu¨ rich, CH-8093 Zu¨ rich, Switzerland Glial cells and neurons are engaged in a continuous and highly Astrocytes, Implications for Myelination and Remyelination Veronica T. Cheli1, J. Correale2, Pablo M. Paez1 and Juana M. Pasquini3 Abstract Iron is a key nutrient for normal central nervous system (CNS) development and function; thus, iron deficiency as well as iron … The galactolipids galactocerebroside and sulfatide and the proteolipid protein (PLP) and its splice variant DM20 are the most abundant lipid and protein components of central nervous system myelin. Recent studies have found that mice lacking either the galactolipids or PLP are able to form myelin sheaths with apparently normal periodicity and near normal compaction. 2002-12-19 · The galactolipids also appear to facilitate the maturation of both oligodendrocytes and myelinated fibers, suggesting that they process developmental signals during myelination.

Numerous studies, mainly using antibody perturbation methods in vitro, have suggested that these molecules are crucial mediators of oligodendrocyte differentiation and myelin formation galactolipids, two glial components implicated in mediating axo-glial interactions during the myelination process. The single-mutant mice produce abnormal myelin containing similar ultrastructural abnormalities, suggesting that these molecules may play an overlapping role in myelin formation. Furthermore, the absence of the galactolipids bodies raised against GalC inhibit myelination in vitro and in vivo31–34. Although these studies suggest a role for GalC in axoglial interactions, the nature of these interactions remains to be determined. Moreover, at T. Coetzee et al.– Myelin galactolipids REVIEW Fig. 1. Galactolipids are a type of glycolipid whose sugar group is galactose.
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gene for the lyso-somal enzyme galactocerebrosidase (GALC) result in low enzymatic activity and decreased ability to degrade galactolipids found in myelin. gene for the lyso-somal enzyme galactocerebrosidase (GALC) result in low enzymatic activity and decreased ability to degrade galactolipids found in myelin. accumulation of galactolipids such as GALACTOSYLCERAMIDES and PSYCHOSINE. The loss of MYELIN disrupts normal conduction of nerve impulses.

The galactolipids galactocerebroside and sulfatide, which require the enzyme UDP‐galactose:ceramide galactosyltransferase (CGT) for their synthesis, are among the most prevalent molecules in the myelin sheath.
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CHANGES DURING EARLY MYELINATION 189 These two measures (Figs. 3 and 4) show very similar developmental profiles in the forebrain as well as in the cerebellum, indicating their value as myelin markers. In the forebrain, galactolipids do not increase their accretion until very near term,

T2 - Dynamics of MBP, PLP and galactolipids. AU - Ozgen, Hande. PY - 2014. Y1 - 2014. N2 - Myelin membrane biogenesis and maintenance require a tight regulation of complex intracellular trafficking machineries, involving appropriate and sequential transportation of major myelin lipids and proteins to their final destination, where they can exert their function. The precise alignment of myelin segments along the length of the axon is essential for the saltatory propagation of an electrical impulse.

Oligodendrocytes (OLs), the myelin-forming cells in the central nervous system, may be a possible target for Pb toxicity. The present study describes the effect of Pb on the maturation of rat OL progenitor (OP) cells and the developmental expression of myelin-specific galactolipids.

2002-12-19 The galactolipids galactocerebroside and sulfatide and the proteolipid protein (PLP) and its splice variant DM20 are the most abundant lipid and protein components of central nervous system myelin. Recent studies have found that mice lacking either the galactolipids or PLP are able to form myelin sheaths with apparently normal periodicity and near normal compaction.

Novel oligodendrocyte transmembrane signaling systems. CHANGES DURING EARLY MYELINATION 189 These two measures (Figs. 3 and 4) show very similar developmental profiles in the forebrain as well as in the cerebellum, indicating their value as myelin markers. In the forebrain, galactolipids do not increase their accretion until very near term, To develop better treatment for these diseases, we must understand the mechanisms involved in myelination. Myelin is a specialized structure with distinct lipid and protein constituents. Galactosylceramide (GalCer) and sulfatide make up approximately 30% of total myelin lipids, and more than half of these galactolipids contain fatty acid with a hydroxyl group at the C2 position (2-OH galactolipids). Myelin in the mammalian nervous system has a high concentration of galactolipids [galactosylceramide (GalCer) and sulfatide] with 2-hydroxy fatty acids.