C1GALT1 transfers Galactose to the Tn antigen forming Core 1 glycoproteins (T antigens)

Stable Identifier
R-HSA-1964505
Type
Reaction [transition]
Species
Homo sapiens
Compartment
Golgi membrane, Golgi lumen
ReviewStatus
5/5
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C1GALT1 transfers Galactose to the Tn antigen forming Core 1 glycoproteins (T antigens)
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Glycoprotein N acetylgalactosamine 3 beta galactosyltransferase 1 (C1GALT1; MIM:610555) mediates the transfer of Galactose (Gal) from UDP galactose to single O-linked GalNAc residues (Tn antigens) to form Core 1 structures on glycoproteins. C1GALT1 is active when in complex with the molecular chaperone C1GALT1C1 (aka COSMC; MIM:300611) which assists the folding and/or stability of C1GALT1. Defects in C1GALT1C1 causes somatic Tn polyagglutination syndrome (TNPS; MIM:300622), characterised by the polyagglutination of erythrocytes by naturally occurring anti Tn antibodies following exposure of the Tn antigen on their surface. Defects in C1GALT1C1 render C1GALT1 inactive and results in the accumulation of the incompletely glycosylated Tn antigen. The Tn antigen is tumour associated, found in a majority of human carcinomas, and is not normally expressed in peripheral tissues or blood cells (Crew et al. 2008, Ju et al. 2014). C1GALT1 and C1GALT1C1 belong to the CAZy family GT31 (CAZy.org).
Literature References
PubMed ID Title Journal Year
24643043 The Cosmc connection to the Tn antigen in cancer

Ju, T, Aryal, RP, Kudelka, MR, Wang, Y, Cummings, RD

Cancer Biomark 2014
18537974 New mutations in C1GALT1C1 in individuals with Tn positive phenotype

Crew, VK, Singleton, BK, Green, C, Parsons, SF, Daniels, G, Anstee, DJ

Br. J. Haematol. 2008
Participants
Input
Output
Participates
as an event of
Catalyst Activity

N-acetylgalactosaminide beta-1,3-galactosyltransferase activity of C1GALT1:C1GALT1C1 [Golgi membrane]

Physical Entity
C1GALT1:C1GALT1C1 [Golgi membrane] (Homo sapiens)
Activity
N-acetylgalactosaminide beta-1,3-galactosyltransferase activity (GO:0016263)
Orthologous Events
C1GALT1 transfers Galactose to the Tn antigen forming Core 1 glycoproteins (T antigens) (Bos taurus)
C1GALT1 transfers Galactose to the Tn antigen forming Core 1 glycoproteins (T antigens) (Caenorhabditis elegans)
C1GALT1 transfers Galactose to the Tn antigen forming Core 1 glycoproteins (T antigens) (Canis familiaris)
C1GALT1 transfers Galactose to the Tn antigen forming Core 1 glycoproteins (T antigens) (Danio rerio)
C1GALT1 transfers Galactose to the Tn antigen forming Core 1 glycoproteins (T antigens) (Drosophila melanogaster)
C1GALT1 transfers Galactose to the Tn antigen forming Core 1 glycoproteins (T antigens) (Gallus gallus)
C1GALT1 transfers Galactose to the Tn antigen forming Core 1 glycoproteins (T antigens) (Mus musculus)
C1GALT1 transfers Galactose to the Tn antigen forming Core 1 glycoproteins (T antigens) (Rattus norvegicus)
C1GALT1 transfers Galactose to the Tn antigen forming Core 1 glycoproteins (T antigens) (Sus scrofa)
C1GALT1 transfers Galactose to the Tn antigen forming Core 1 glycoproteins (T antigens) (Xenopus tropicalis)
Cross References
RHEA
Authored
Jassal, B  (2010-07-19)
Reviewed
Ferrer, A  (2011-11-04)
D'Eustachio, P  (2025-04-08)
Created
Jassal, B  (2011-11-04)
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