GalCer Synthase (Ceramide Galactosyltransferase, CGT)

  • Wilhelm Stoffel


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).


Schwann Cell Myelin Membrane Myelin Lipid Saltatory Conduction Ceramide Galactosyltransferase 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Basu S, Kaufman B, Roseman SJ (1968) Enzymatic synthesis of ceramide glucose and ceramide lactose by glycosyltransferases from embryonic chicken brain. J Biol Chem 243:5802–5804PubMedGoogle Scholar
  2. 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 93:13280–13285PubMedCrossRefGoogle Scholar
  3. Bosio A, Binczek E, Stoffel W (1996b) Molecular cloning and characterization of the mouse CGT gene encoding UDP-galactose ceramide-galactosyltransferase (cerebroside synthetase). Genomics 35:223–226PubMedCrossRefGoogle Scholar
  4. 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 70:308–315PubMedCrossRefGoogle Scholar
  5. 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 70:308–315PubMedCrossRefGoogle Scholar
  6. Brady RO (1962) Studies on the total enzymatic synthesis of cerebrosides. J Biol Chem 237:2416–2417PubMedGoogle Scholar
  7. Clealand WW, Kennedy EP (1960) The enzymatic synthesis of psychosine. J Biol Chem 235:45–51Google Scholar
  8. 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 86:209–219PubMedCrossRefGoogle Scholar
  9. 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 35:215–222PubMedCrossRefGoogle Scholar
  10. Gossen M, Bujard H (1992) Tight control of gene expression in mammalian cells by tetracyclin-responsive promoters. Proc Natl Acad Sci USA 89:5547–5551PubMedCrossRefGoogle Scholar
  11. Kopaczyk KC, Radin NS (1965) In vivo conversion of cerebrosides and ceramide in rat brain. J Lipid Res 6:140–148PubMedGoogle Scholar
  12. Morell P, Radin NS (1969) Synthesis of cerebroside by brain from uridine diphosphate galactose-and ceramide-containing hydroxy fatty acid. Biochemistry 8:506–513PubMedCrossRefGoogle Scholar
  13. Moser HM, Karnovsky ML (1959) Studies on the biosynthesis of glycolipids and other lipids of the brain. J Biol Chem 234:1990–1997PubMedGoogle Scholar
  14. Nescovic NM, Sarlieve LL, Mandel P (1974) Purification and properties of UDP-galactosexeramide galactosyltransferase from rat brain microsomes. Biochem Biophys Acta 334:309–315CrossRefGoogle Scholar
  15. Nescovic NM, Mandel P, Gatt S (1981) UDP-Galactose: (2-hydroxyacyl) sphingosine galactosyltransferase from rat brain. Methods Enzymol 71:321–327Google Scholar
  16. 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 47:1412–1418PubMedCrossRefGoogle Scholar
  17. Ong D, Brady R (1972) Synthesis of ceramides using N-hydroxysuccinimide esters. J Lipid Res 13:819–822PubMedGoogle Scholar
  18. Sauer B, Henderson N (1988) Site-specific DANN recombination in mammalian cells by the Cre recombinase of bacteriophage P1. Proc Natl Acad Sci USA 85:166–170CrossRefGoogle Scholar
  19. Schulte S, Stoffel W (1993) Ceramide UDP-galactosyltransferase from myelinating rat brain: purification, cloning, and expression. Proc Natl Acad Sci USA 90:10265–10269PubMedCrossRefGoogle Scholar
  20. Schulte S, Stoffel W (1995) UDP-galactose:ceramide galactosyltransferase and glutamate/aspartate transporter. Copurification, separation and characterization of the two glycoproteins. Eur J Biochem 233:947–953PubMedCrossRefGoogle Scholar

Copyright information

© Springer Japan 2002

Authors and Affiliations

  • Wilhelm Stoffel
    • 1
  1. 1.Laboratory of Molecular Neuroscience, Institute of Biochemistry, Faculty of MedicineUniversity of CologneKölnGermany

Personalised recommendations