A defining feature of the vertebrate nervous system is the ensheathment of axons by myelin, a multilamellar membrane containing a small group of proteins and an abundance of the galactolipid galactocerebroside (GalC) and its sulfated derivative sulfatide.

Myelin in the mammalian nervous system has a high concentration of galactolipids [galactosylceramide (GalCer) and sulfatide] with 2-hydroxy fatty acids. We recently reported that fatty acid 2-hydroxylase (FA2H), encoded by the FA2H gene, is the major fatty acid 2-hydroxylase in the mouse brain. In this report, we show that FA2H also plays a major

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

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(1999). Myelination in the absence of galactolipids and proteolipid proteins. (1994). Myelination in the absence of myelin-associated glycoprotein.

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

We recently reported that fatty acid 2-hydroxylase (FA2H), encoded by the FA2H gene, is the major fatty acid 2-hydroxylase in the mouse brain. In this report, we show that FA2H also plays a major transition of galactolipid molecular species during myelin- In both CNS and PNS, 2-hydroxy fatty acid contents in ation are likely achieved by the coordinated regulation of galactolipids are very low at the onset of myelination and specific Elovl gene(s) and Lass/CerS gene(s) along with FA2H. increase rapidly during developmental myelination. CHANGES DURING EARLY MYELINATION 189 These two measures (Figs.

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.

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. They differ from glycosphingolipids in that they do not have nitrogen in their composition. They are the main part of plant membrane lipids where they substitute phospholipids to conserve phosphate for other essential processes. 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.

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.
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Download Citation | Galactolipids are molecular determinants of myelin development and axo-glial organization | Myelination is a developmentally regulated process whereby myelinating glial cells A defining feature of the vertebrate nervous system is the ensheathment of axons by myelin, a multilamellar membrane containing a small group of proteins and an abundance of the galactolipid galactocerebroside (GalC) and its sulfated derivative sulfatide. In this study, we have tested for the possibility that MAG and myelin galactolipids function similarly in mediating early stages of myelination. Previous experiments, mainly cell culture based, have employed biochemical strategies to determine that MAG and the galactolipids are critical early mediators of myelin formation ( Dupree et al., 1998c ; Schachner and Bartsch, 2000 ). Myelin-Associated Galactolipids in Primary Cultures from Dissociated Fetal Rat Brain: Biosynthesis, Accumulation, and Cell Surface Expression Martínez M. The developmental lipid profiles in the human cerebrum, cerebellum and brain stem are presented, with special reference to galactolipids as myelin markers to trace myelination in the three main parts of the human CNS. A group of undernourished children were also studied to test the vulnerability of myelinogenesis in the different 1999-07-01 · 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.

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.
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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.

Read "Myelin‐Associated Galactolipids in Primary Cultures from Dissociated Fetal Rat Brain: Biosynthesis, Accumulation, and Cell Surface Expression, Journal of Neurochemistry" on DeepDyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. The fatty acid compositions of galactolipids in neonatal mouse brain gradually changed during the course of myelination. The relative ratio of 2-hydroxy versus nonhydroxy galactolipids was very low at 2 days of age (∼8% of total galactolipids) and increased 6- to 8-fold by 30 days of age. Lipid hydroxylation in glial cell signaling and myelination Hama, Hiroko Medical University of South Carolina, Charleston, SC, United States.

gene for the lyso-somal enzyme galactocerebrosidase (GALC) result in low enzymatic activity and decreased ability to degrade galactolipids found in myelin.

Brain slices from 14‐, 17‐, and 21‐day‐old D2, B6, and F., mice were incubated with [ 3 H]galactose and [ 35 S]sulfate. Approximately 70% of the dry weight of myelin is lipid, with the galactolipid galactocerebroside (GalC) and its sulfated derivative sulfatide encompassing about one-third of the lipid mass (reviewed by Norton and Cammer, 1984).

This dense network of hydrogen bonds is believed to contribute to the high transition temperature and the compact alignment of the galactolipids.