EC 2.4.1.47 - N-acylsphingosine galactosyltransferase

  IntEnz view ENZYME view

IntEnz Enzyme Nomenclature
EC 2.4.1.47

Names

Accepted name:
N-acylsphingosine galactosyltransferase
Other names:
UDP galactose-N-acylsphingosine galactosyltransferase
UDPgalactose:N-acylsphingosine D-galactosyltransferase
uridine diphosphogalactose-acylsphingosine galactosyltransferase
UDP-galactose:N-acylsphingosine D-galactosyltransferase
UDP-α-D-galactose:N-acylsphingosine D-galactosyltransferase
UGT8 (gene name)
CGT (gene name)
2-hydroxyacylsphingosine 1-β-galactosyltransferase
Systematic name:
UDP-α-D-galactose:N-acylsphingosine β-D-galactosyltransferase (configuration-inverting)

Reactions

Comments:

This membrane-bound, endoplasmic reticulum-located enzyme catalyses the last step in the synthesis of galactocerebrosides, which are abundant sphingolipids of the myelin membrane of the central nervous system and peripheral nervous system. It has a strong preference for ceramides that contain hydroxylated fatty acids. Formerly EC 2.4.1.45.

Links to other databases

Enzymes and pathways: NC-IUBMB , BRENDA , ERGO , ExplorEnz , ENZYME@ExPASy , KEGG , MetaCyc , UniPathway
Structural data: CSA , EC2PDB
Gene Ontology: GO:0003851 , GO:0047263
CAS Registry Number: 37277-56-8
UniProtKB/Swiss-Prot:

References

  1. Fujino, Y. and Nakano, M.
    Enzymic synthesis of cerebroside from ceramide and uridine diphosphate galactose.
    Biochem. J. 113 : 573-575 (1969). [PMID: 5807218]
  2. Morell, P., Radin, N. S.
    Synthesis of cerebroside by brain from uridine diphosphate galactose and ceramide containing hydroxy fatty acid.
    Biochemistry 8 : 506-512 (1969). [PMID: 5793706]
  3. Morell, P., Costantino-Ceccarini, E., Radin, N. S.
    The biosynthesis by brain microsomes of cerebrosides containing nonhydroxy fatty acids.
    Arch. Biochem. Biophys. 141 : 738-748 (1970). [PMID: 5497154]
  4. Basu, S., Schultz, A. M., Basu, M., Roseman, S.
    Enzymatic synthesis of galactocerebroside by a galactosyltransferase from embryonic chicken brain.
    J. Biol. Chem. 246 : 4272-4279 (1971). [PMID: 5090043]
  5. Akanuma, H., Kishimoto, Y.
    Synthesis of ceramides and cerebrosides containing both alpha-hydroxy and nonhydroxy fatty acids from lignoceroyl-CoA by rat brain microsomes.
    J. Biol. Chem. 254 : 1050-1060 (1979). [PMID: 762114]
  6. Koul, O., Jungalwala, F. B.
    UDP-galactose:ceramide galactosyltransferase of rat central-nervous-system myelin.
    Biochem. J. 194 : 633-637 (1981). [PMID: 7306007]
  7. Schulte, S., Stoffel, W.
    Ceramide UDPgalactosyltransferase from myelinating rat brain: purification, cloning, and expression.
    Proc. Natl. Acad. Sci. U.S.A. 90 : 10265-10269 (1993). [PMID: 7694285]
  8. Sprong, H., Kruithof, B., Leijendekker, R., Slot, J. W., van Meer, G., van der Sluijs, P.
    UDP-galactose:ceramide galactosyltransferase is a class I integral membrane protein of the endoplasmic reticulum.
    J. Biol. Chem. 273 : 25880-25888 (1998). [PMID: 9748263]
  9. Fewou, S. N., Bussow, H., Schaeren-Wiemers, N., Vanier, M. T., Macklin, W. B., Gieselmann, V., Eckhardt, M.
    Reversal of non-hydroxy:alpha-hydroxy galactosylceramide ratio and unstable myelin in transgenic mice overexpressing UDP-galactose:ceramide galactosyltransferase.
    J. Neurochem. 94 : 469-481 (2005). [PMID: 15998297]

[EC 2.4.1.47 created 1972]