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GalCer Synthase (Ceramide Galactosyltransferase, CGT)

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Handbook of Glycosyltransferases and Related Genes

Abstract

The multilayer membrane system of myelin ensheathing axons of the central nervous system (CNS) and the peripheral nervous system (PNS) is synthesized and assembled by oligodendrocytes and Schwann cells, respectively. The lipid bilayer contributes approximately 70% to 85% of the total myelin mass. The lipid composition is characterized by a high content of cholesterol and glycolipids, of which galactosylceramides (cerebrosides, GalC) and sulfatides, the 3′-sulfoesters of galactosyl-ceramides (sGalC), have been recognized as the dominant components and also oligodendrocyte-speciflc myelin markers. Both are expressed in only a minute amount in kidney. The molar ratio of cholesterohphospholipids (including plasmalogen-phospholipids):galactolipids is between 2:2:1 and 4:3:2 and reasonably stable in different species (human, bovine, and murine myelin).

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References

  • Basu S, Kaufman B, Roseman SJ (1968) Enzymatic synthesis of ceramide glucose and ceramide lactose by glycosyltransferases from embryonic chicken brain. J Biol Chem 243:5802–5804

    PubMed  CAS  Google Scholar 

  • Bosio A, Binczek E, Stoffel W (1996a) Functional breakdown of the lipid bilayer of the myelin membrane in central and peripheral nervous system by disrupted galactocerebroside synthesis. Proc Natl Acad Sci USA 93:13280–13285

    Article  PubMed  CAS  Google Scholar 

  • Bosio A, Binczek E, Stoffel W (1996b) Molecular cloning and characterization of the mouse CGT gene encoding UDP-galactose ceramide-galactosyltransferase (cerebroside synthetase). Genomics 35:223–226

    Article  PubMed  CAS  Google Scholar 

  • Bosio A, Binczek E, Haupt WF, Stoffel W (1998a) Composition and biophysical properties of myelin lipid define the neurological defects in galactocerebroside-sulfatide-deficient mice. J Neurochem 70:308–315

    Article  PubMed  CAS  Google Scholar 

  • Bosio A, Binczek E, Haupt WF, Stoffel W (1998b) Biophysical properties of myelin lipid define the neurological defects in galactocerebroside-and sulfatide-deficient mice. J Neurochem 70:308–315

    Article  PubMed  CAS  Google Scholar 

  • Brady RO (1962) Studies on the total enzymatic synthesis of cerebrosides. J Biol Chem 237:2416–2417

    PubMed  CAS  Google Scholar 

  • Clealand WW, Kennedy EP (1960) The enzymatic synthesis of psychosine. J Biol Chem 235:45–51

    Google Scholar 

  • Coetzee T, Fujita N, Dupree J, Shi R, Blight A, Suzuki K, Popko B (1996a) Myelination in the absence of galactocerebroside and sulfatide: normal structure with abnormal function and regional instability. Cell 86:209–219

    Article  PubMed  CAS  Google Scholar 

  • Coetzee T, Li X, Fujita N, Marcus J, Suzuki K, Francke U, Popko B (1996b) Molecular cloning, chromosomal mapping, and characterization of the mouse UDP-galactose:ceramide galactosyltransferase gene. Genomics 35:215–222

    Article  PubMed  CAS  Google Scholar 

  • Gossen M, Bujard H (1992) Tight control of gene expression in mammalian cells by tetracyclin-responsive promoters. Proc Natl Acad Sci USA 89:5547–5551

    Article  PubMed  CAS  Google Scholar 

  • Kopaczyk KC, Radin NS (1965) In vivo conversion of cerebrosides and ceramide in rat brain. J Lipid Res 6:140–148

    PubMed  CAS  Google Scholar 

  • Morell P, Radin NS (1969) Synthesis of cerebroside by brain from uridine diphosphate galactose-and ceramide-containing hydroxy fatty acid. Biochemistry 8:506–513

    Article  PubMed  CAS  Google Scholar 

  • Moser HM, Karnovsky ML (1959) Studies on the biosynthesis of glycolipids and other lipids of the brain. J Biol Chem 234:1990–1997

    PubMed  CAS  Google Scholar 

  • Nescovic NM, Sarlieve LL, Mandel P (1974) Purification and properties of UDP-galactosexeramide galactosyltransferase from rat brain microsomes. Biochem Biophys Acta 334:309–315

    Article  Google Scholar 

  • Nescovic NM, Mandel P, Gatt S (1981) UDP-Galactose: (2-hydroxyacyl) sphingosine galactosyltransferase from rat brain. Methods Enzymol 71:321–327

    Google Scholar 

  • Neskovic N, Roussel G, Nussbaum J (1986) UDP-galactose:ceramide galactosyltransferase of rat brain: a new method of purification and production of specific antibodies. J Neurochem 47:1412–1418

    Article  PubMed  CAS  Google Scholar 

  • Ong D, Brady R (1972) Synthesis of ceramides using N-hydroxysuccinimide esters. J Lipid Res 13:819–822

    PubMed  CAS  Google Scholar 

  • Sauer B, Henderson N (1988) Site-specific DANN recombination in mammalian cells by the Cre recombinase of bacteriophage P1. Proc Natl Acad Sci USA 85:166–170

    Article  Google Scholar 

  • Schulte S, Stoffel W (1993) Ceramide UDP-galactosyltransferase from myelinating rat brain: purification, cloning, and expression. Proc Natl Acad Sci USA 90:10265–10269

    Article  PubMed  CAS  Google Scholar 

  • Schulte S, Stoffel W (1995) UDP-galactose:ceramide galactosyltransferase and glutamate/aspartate transporter. Copurification, separation and characterization of the two glycoproteins. Eur J Biochem 233:947–953

    Article  PubMed  CAS  Google Scholar 

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© 2002 Springer Japan

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Stoffel, W. (2002). GalCer Synthase (Ceramide Galactosyltransferase, CGT). In: Taniguchi, N., et al. Handbook of Glycosyltransferases and Related Genes. Springer, Tokyo. https://doi.org/10.1007/978-4-431-67877-9_8

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  • DOI: https://doi.org/10.1007/978-4-431-67877-9_8

  • Publisher Name: Springer, Tokyo

  • Print ISBN: 978-4-431-67996-7

  • Online ISBN: 978-4-431-67877-9

  • eBook Packages: Springer Book Archive

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