Names & Taxonomy

Uniprot ID:
Q9P2W7
Entry Name:
B3GA1_HUMAN
Status:
reviewed
Protein Names:
Galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 1 (EC 2.4.1.135) (Beta-1,3-glucuronyltransferase 1) (Glucuronosyltransferase P) (GlcAT-P) (UDP-GlcUA:glycoprotein beta-1,3-glucuronyltransferase) (GlcUAT-P)
Gene Names:
B3GAT1 GLCATP
Gene Names Primary:
B3GAT1
Organism:
Homo sapiens (Human)

Structure

Length:
334
Sequence:
MPKRRDILAIVLIVLPWTLLITVWHQSTLAPLLAVHKDEGSDPRRETPPGADPREYCTSDRDIVEVVRTEYVYTRPPPWSDTLPTIHVVTPTYSRPVQKAELTRMANTLLHVPNLHWLVVEDAPRRTPLTARLLRDTGLNYTHLHVETPRNYKLRGDARDPRIPRGTMQRNLALRWLRETFPRNSSQPGVVYFADDDNTYSLELFEEMRSTRRVSVWPVAFVGGLRYEAPRVNGAGKVVGWKTVFDPHRPFAIDMAGFAVNLRLILQRSQAYFKLRGVKGGYQESSLLRELVTLNDLEPKAANCTKILVWHTRTEKPVLVNEGKKGFTDPSVEI
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Golgi apparatus membrane; Single-pass type II membrane protein.

Function

Function:
Involved in the biosynthesis of L2/HNK-1 carbohydrate epitope on glycoproteins. Can also play a role in glycosaminoglycan biosynthesis. Substrates include asialo-orosomucoid (ASOR), asialo-fetuin, and asialo-neural cell adhesion molecule. Requires sphingomyelin for activity: stearoyl-sphingomyelin was the most effective, followed by palmitoyl-sphingomyelin and lignoceroyl-sphingomyelin. Activity was demonstrated only for sphingomyelin with a saturated fatty acid and not for that with an unsaturated fatty acid, regardless of the length of the acyl group (By similarity).
Pathway:
Protein modification; protein glycosylation.
Catalytic Activity:
UDP-glucuronate + 3-beta-D-galactosyl-4-beta-D-galactosyl-O-beta-D-xylosylprotein = UDP + 3-beta-D-glucuronosyl-3-beta-D-galactosyl-4-beta-D-galactosyl-O-beta-D-xylosylprotein.
Cofactor:
COFACTOR: Name=Mn(2+); Xref=ChEBI:CHEBI:29035;
Active Site:
ACT_SITE 284 284 Proton donor/acceptor.
Gene Ontology Go:
Golgi membrane
integral component of membrane
galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase activity
metal ion binding
carbohydrate metabolic process
chondroitin sulfate metabolic process
chondroitin sulfate proteoglycan biosynthetic process
glycosaminoglycan metabolic process
protein glycosylation
small molecule metabolic process
Gene Ontology Biological Process:
carbohydrate metabolic process
chondroitin sulfate metabolic process
chondroitin sulfate proteoglycan biosynthetic process
glycosaminoglycan metabolic process
protein glycosylation
small molecule metabolic process
Gene Ontology Molecular Function:
galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase activity
metal ion binding
Gene Ontology Cellular Component:
Golgi membrane
integral component of membrane
Keywords:
3D-structure
Complete proteome
Glycoprotein
Golgi apparatus
Manganese
Membrane
Metal-binding
Phosphoprotein
Polymorphism
Reference proteome
Signal-anchor
Transferase
Transmembrane
Transmembrane helix

Publication

PubMed ID:
10783264 15489334 14993226