(N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase

From Wikipedia, the free encyclopedia
Jump to navigation Jump to search
(N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase
EC number
CAS number 67338-98-1
IntEnz IntEnz view
ExPASy NiceZyme view
MetaCyc metabolic pathway
PRIAM profile
PDB structures RCSB PDB PDBe PDBsum

(N-acetylneuraminyl)-galactosylglucosylceramide N-acetylgalactosaminyltransferase (EC, uridine diphosphoacetylgalactosamine-ganglioside GM3 acetylgalactosaminyltransferase, ganglioside GM2 synthase, ganglioside GM3 acetylgalactosaminyltransferase, GM2 synthase, UDP acetylgalactosamine-(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide acetylgalactosaminyltransferase, UDP-N-acetyl-D-galactosamine:1-O-[O-(N-acetyl-alpha-neuraminyl)-(2->3)-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl]-ceramide 1,4-beta-N-acetyl-D-galactosaminyltransferase acetylgalactosaminyltransferase, UDP-N-acetylgalactosamine GM3 N-acetylgalactosaminyltransferase, uridine diphosphoacetylgalactosamine-acetylneuraminylgalactosylglucosylceramide acetylgalactosaminyltransferase, uridine diphosphoacetylgalactosamine-hematoside acetylgalactosaminyltransferase, GM2/GD2-synthase, beta-1,4N-aetylgalactosaminyltransferase, asialo-GM2 synthase, GalNAc-T, UDP-N-acetyl-D-galactosamine:(N-acetylneuraminyl)-D-galactosyl-D-glucosylceramide N-acetyl-D-galactosaminyltransferase) is an enzyme with systematic name UDP-N-acetyl-D-galactosamine:1-O-(O-(N-acetyl-alpha-neuraminyl)-(2->3)-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl)-ceramide 4-beta-N-acetyl-D-galactosaminyltransferase.[1][2][3][4][5][6][7] This enzyme catalyses the following chemical reaction

UDP-N-acetyl-D-galactosamine + 1-O-[O-(N-acetyl-alpha-neuraminyl)-(2->3)-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl]-ceramide UDP + 1-O-[O-2-(acetylamino)-2-deoxy-beta-D-galactopyranosyl-(1->4)-O-[N-acetyl-alpha-neuraminyl-(2->3)]-O-beta-D-galactopyranosyl-(1->4)-beta-D-glucopyranosyl]-ceramide

This enzyme catalyses the formation of the gangliosides (i.e. sialic-acid-containing glycosphingolipids) GM2, GD2 and SM2 from GM3, GD3 and SM3, respectively.


  1. ^ Dicesare JL, Dain JA (March 1971). "The enzymic synthesis of ganglioside. IV. UDP-N-acetylgalactosamine: (N-acetylneuraminyl)-galactosylglucosyl ceramide N-acetylgalactosaminyltransferase in rat brain". Biochimica et Biophysica Acta. 231 (2): 385–93. doi:10.1016/0005-2760(71)90151-2. PMID 5554906. 
  2. ^ Pohlentz G, Klein D, Schwarzmann G, Schmitz D, Sandhoff K (October 1988). "Both GA2, GM2, and GD2 synthases and GM1b, GD1a, and GT1b synthases are single enzymes in Golgi vesicles from rat liver". Proceedings of the National Academy of Sciences of the United States of America. 85 (19): 7044–8. doi:10.1073/pnas.85.19.7044. PMC 282120Freely accessible. PMID 3140234. 
  3. ^ Hidari JK, Ichikawa S, Furukawa K, Yamasaki M, Hirabayashi Y (November 1994). "beta 1-4N-acetylgalactosaminyltransferase can synthesize both asialoglycosphingolipid GM2 and glycosphingolipid GM2 in vitro and in vivo: isolation and characterization of a beta 1-4N-acetylgalactosaminyltransferase cDNA clone from rat ascites hepatoma cell line AH7974F". The Biochemical Journal. 303 ( Pt 3): 957–65. PMC 1137639Freely accessible. PMID 7980468. 
  4. ^ Hashimoto Y, Sekine M, Iwasaki K, Suzuki A (December 1993). "Purification and characterization of UDP-N-acetylgalactosamine GM3/GD3 N-acetylgalactosaminyltransferase from mouse liver". The Journal of Biological Chemistry. 268 (34): 25857–64. PMID 8245020. 
  5. ^ Nagai K, Ishizuka I (May 1987). "Biosynthesis of monosulfogangliotriaosylceramide and GM2 by N-acetylgalactosaminyltransferase from rat brain". Journal of Biochemistry. 101 (5): 1115–27. PMID 3115968. 
  6. ^ Furukawa K, Takamiya K, Furukawa K (December 2002). "Beta1,4-N-acetylgalactosaminyltransferase--GM2/GD2 synthase: a key enzyme to control the synthesis of brain-enriched complex gangliosides". Biochimica et Biophysica Acta. 1573 (3): 356–62. doi:10.1016/s0304-4165(02)00403-8. PMID 12417418. 
  7. ^ Yamashita T, Wu YP, Sandhoff R, Werth N, Mizukami H, Ellis JM, Dupree JL, Geyer R, Sandhoff K, Proia RL (February 2005). "Interruption of ganglioside synthesis produces central nervous system degeneration and altered axon-glial interactions". Proceedings of the National Academy of Sciences of the United States of America. 102 (8): 2725–30. doi:10.1073/pnas.0407785102. PMC 549464Freely accessible. PMID 15710896. 

External links[edit]