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beta-1,3-glucuronyltransferase 3

Summary

Gene Symbol

B3GAT3

Aliases

GlcAT-I
galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3
glucuronosyltransferase I

Organism

Homo sapiens (human)

External Links

NCBI Gene

26229

GGDB ID

gg155

HGNC

923

mRNA

NM_012200

map

11q12.3

Protein

NP_036332

OMIM

606374

KEGG Gene ID

hsa:26229

PubChem

26229

Alliance of Genome Resources

HGNC:923

Annotation

Keyword

3D-structure
Alternative splicing
Disease variant
Disulfide bond
Glycoprotein
Golgi apparatus
Manganese
Metal-binding
Proteomics identification
Reference proteome
Signal-anchor
Transferase
Transmembrane helix

Proteins

Displaying **all 3** entries
UniProt	Protein Name
G3V150
Q5U676
O94766	Beta-1,3-glucuronyltransferase 3 Glucuronosyltransferase I UDP-GlcUA:Gal beta-1,3-Gal-R glucuronyltransferase

Annotation information from UniProt.

Gene Ontology (GO)

Displaying entries **1 - 5** of 14 in total

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GO Term	Evidence Code	PMID
carbohydrate metabolic process	IBA
glycosaminoglycan biosynthetic process	NAS	10842173
glycosaminoglycan biosynthetic process	IDA	24425863
glycosaminoglycan biosynthetic process	IMP	25893793
protein glycosylation	IEA

GO Hierarchy

biological process

metabolic process [inference]

nitrogen compound metabolic process [inference]

organonitrogen compound metabolic process [inference]

aminoglycan metabolic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan biosynthetic process

protein metabolic process [inference]

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan biosynthetic process [inference]

chondroitin sulfate proteoglycan metabolic process [inference]

chondroitin sulfate proteoglycan biosynthetic process

dermatan sulfate proteoglycan metabolic process [inference]

dermatan sulfate proteoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

protein modification process [inference]

protein glycosylation

organonitrogen compound biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

biosynthetic process [inference]

cellular biosynthetic process [inference]

macromolecule biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

sulfur compound biosynthetic process [inference]

heparan sulfate proteoglycan biosynthetic process

organic substance biosynthetic process [inference]

macromolecule biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

carbohydrate derivative biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

organonitrogen compound biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

cellular metabolic process [inference]

sulfur compound metabolic process [inference]

sulfur compound biosynthetic process [inference]

heparan sulfate proteoglycan biosynthetic process

cellular biosynthetic process [inference]

macromolecule biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

sulfur compound biosynthetic process [inference]

heparan sulfate proteoglycan biosynthetic process

primary metabolic process [inference]

carbohydrate metabolic process

protein metabolic process [inference]

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan biosynthetic process [inference]

chondroitin sulfate proteoglycan metabolic process [inference]

chondroitin sulfate proteoglycan biosynthetic process

dermatan sulfate proteoglycan metabolic process [inference]

dermatan sulfate proteoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

protein modification process [inference]

protein glycosylation

glycosylation [inference]

macromolecule glycosylation [inference]

protein glycosylation

organic substance metabolic process [inference]

carbohydrate metabolic process

macromolecule metabolic process [inference]

aminoglycan metabolic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan biosynthetic process

macromolecule biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

protein metabolic process [inference]

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan biosynthetic process [inference]

chondroitin sulfate proteoglycan metabolic process [inference]

chondroitin sulfate proteoglycan biosynthetic process

dermatan sulfate proteoglycan metabolic process [inference]

dermatan sulfate proteoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

protein modification process [inference]

protein glycosylation

macromolecule modification [inference]

protein modification process [inference]

protein glycosylation

macromolecule glycosylation [inference]

protein glycosylation

carbohydrate derivative metabolic process [inference]

aminoglycan metabolic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan biosynthetic process [inference]

chondroitin sulfate proteoglycan metabolic process [inference]

chondroitin sulfate proteoglycan biosynthetic process

dermatan sulfate proteoglycan metabolic process [inference]

dermatan sulfate proteoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

carbohydrate derivative biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

organonitrogen compound metabolic process [inference]

aminoglycan metabolic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan biosynthetic process

protein metabolic process [inference]

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan biosynthetic process [inference]

chondroitin sulfate proteoglycan metabolic process [inference]

chondroitin sulfate proteoglycan biosynthetic process

dermatan sulfate proteoglycan metabolic process [inference]

dermatan sulfate proteoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

protein modification process [inference]

protein glycosylation

organonitrogen compound biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

organic substance biosynthetic process [inference]

macromolecule biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

carbohydrate derivative biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

organonitrogen compound biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

cellular process [inference]

cellular metabolic process [inference]

sulfur compound metabolic process [inference]

sulfur compound biosynthetic process [inference]

heparan sulfate proteoglycan biosynthetic process

cellular biosynthetic process [inference]

macromolecule biosynthetic process [inference]

aminoglycan biosynthetic process [inference]

glycosaminoglycan biosynthetic process

glycoprotein biosynthetic process [inference]

proteoglycan biosynthetic process [inference]

sulfur compound biosynthetic process [inference]

heparan sulfate proteoglycan biosynthetic process

biological regulation [inference]

regulation of biological process [inference]

regulation of localization [inference]

regulation of transport [inference]

regulation of intracellular transport [inference]

positive regulation of intracellular transport [inference]

positive regulation of intracellular protein transport

regulation of intracellular protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of transport [inference]

positive regulation of intracellular transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

regulation of protein transport [inference]

regulation of intracellular protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

regulation of cellular localization [inference]

regulation of intracellular transport [inference]

positive regulation of intracellular transport [inference]

positive regulation of intracellular protein transport

regulation of intracellular protein transport [inference]

positive regulation of intracellular protein transport

regulation of protein localization [inference]

regulation of establishment of protein localization [inference]

regulation of protein transport [inference]

regulation of intracellular protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of establishment of protein localization [inference]

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein localization [inference]

positive regulation of establishment of protein localization [inference]

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of biological process [inference]

positive regulation of cellular process [inference]

positive regulation of intracellular transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein localization [inference]

positive regulation of establishment of protein localization [inference]

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of transport [inference]

positive regulation of intracellular transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

regulation of cellular process [inference]

positive regulation of cellular process [inference]

positive regulation of intracellular transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein localization [inference]

positive regulation of establishment of protein localization [inference]

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

regulation of cellular localization [inference]

regulation of intracellular transport [inference]

positive regulation of intracellular transport [inference]

positive regulation of intracellular protein transport

regulation of intracellular protein transport [inference]

positive regulation of intracellular protein transport

regulation of protein localization [inference]

regulation of establishment of protein localization [inference]

regulation of protein transport [inference]

regulation of intracellular protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of establishment of protein localization [inference]

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

positive regulation of protein localization [inference]

positive regulation of establishment of protein localization [inference]

positive regulation of protein transport [inference]

positive regulation of intracellular protein transport

regulation of molecular function [inference]

positive regulation of molecular function [inference]

positive regulation of catalytic activity

regulation of catalytic activity [inference]

positive regulation of catalytic activity

Displaying entries **1 - 5** of 7 in total

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GO Term	Evidence Code	PMID
Golgi membrane	TAS
Golgi membrane	IBA
Golgi apparatus	IDA	21763480
cis-Golgi network	IDA	21763480 25893793
membrane	NAS	10842173

GO Hierarchy

cellular component

cellular anatomical entity [inference]

membrane

organelle membrane [inference]

bounding membrane of organelle [inference]

Golgi membrane

organelle [inference]

membrane-bounded organelle [inference]

vesicle [inference]

extracellular vesicle [inference]

extracellular exosome

intracellular membrane-bounded organelle [inference]

extracellular membrane-bounded organelle [inference]

extracellular vesicle [inference]

extracellular exosome

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

extracellular organelle [inference]

extracellular membrane-bounded organelle [inference]

extracellular vesicle [inference]

extracellular exosome

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GO Term	Evidence Code	PMID
protein binding	IPI	24425863 32707033
galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase activity	NAS	10842173
galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase activity	IBA
glucuronosyltransferase activity	IDA	25893793
glucuronosyltransferase activity	IMP	21763480

GO Hierarchy

molecular function

catalytic activity [inference]

transferase activity [inference]

glycosyltransferase activity [inference]

UDP-glycosyltransferase activity [inference]

glucuronosyltransferase activity

galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase activity

hexosyltransferase activity [inference]

glucuronosyltransferase activity

galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase activity

binding [inference]

protein binding

ion binding [inference]

cation binding [inference]

metal ion binding

molecular function regulator activity [inference]

enzyme regulator activity [inference]

enzyme activator activity [inference]

phosphatase activator activity [inference]

protein phosphatase activator activity

phosphatase regulator activity [inference]

phosphatase activator activity [inference]

protein phosphatase activator activity

protein phosphatase regulator activity [inference]

protein phosphatase activator activity

molecular function activator activity [inference]

enzyme activator activity [inference]

phosphatase activator activity [inference]

protein phosphatase activator activity

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

Galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase S

Functional Category

E: Amino acid transport and metabolism
O: Posttranslational modification, protein turnover, chaperones
T: Signal transduction mechanisms

Displaying **all 4** entries
Gene Ontology
chondroitin sulfate proteoglycan biosynthetic process
galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase activity
metal ion binding
transferase activity

Displaying **all 2** entries
InterPro
Glycosyl transferase, family 43
Nucleotide-diphospho-sugar transferases

Human Protein Atlas

ENSG00000149541

Tissue with high expression from Human Protein Atlas. Tissues that are highly expressed are highlighted.

Data available from v23.0.proteinatlas.org. Image credit: Human Protein Atlas

GlycoGene Database (GGDB)

GGDB ID

gg155

Gene Symbol

B3GAT3

Reactions

Displaying 1 entry
Donor	Acceptor	Reducing terminal(Acceptor)	Product	Reducing terminal(Product)	Reference
UDP-GlcUA	G87877ES	O-Ser	G14371GV	O-Ser	9506957

Displaying 1 entry
Donor	Acceptor	Reducing terminal(Acceptor)	Product	Reducing terminal(Product)	Reference
UDP-GlcUA	G87877ES	O-Ser	G14371GV	O-Ser	9506957

Displaying **all 2** entries
Donor	Acceptor	Reducing terminal(Acceptor)	Reference
UDP-GlcUA	G40609EP	O-Ser	9506957
UDP-GlcUA	G95036CM	O-Ser	9506957

Orthologous Gene

Displaying **all 3** entries
Species	Protein	mRNA
Rattus norvegicus	XP_238155	XM_238155
Mus musculus	NP_077218	NM_024256
Drosophila melanogaster	NP_726910	NM_167003

KEGG BRITE Database

Orthology

K10158

Name

galactosylgalactosylxylosylprotein 3-beta-glucuronosyltransferase 3 [EC:2.4.1.135]

References

Reactions

Displaying 1 entry
KEGG Reaction	Enzyme	Acceptor	Product
R05928	2.4.1.135	G87877ES	G14371GV

Rhea

RHEA:24168

UDP-α-D-glucuronate

H⁺ + UDP

Enzyme

2.4.1.135

PMID

RHEA:56508

UDP-α-D-glucuronate

H⁺ + UDP

Enzyme

2.4.1.135

PMID

18400750

Disease

Disease Ontology

Displaying 1 entry
DO ID	Disease Name	Source
DOID:0080575	Larsen-like syndrome B3GAT3 type	Glyco-Disease Genes Database Alliance of Genome Resources

The Human Phenotype Ontology

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HPO ID	HPO Term
HP:0000007	Autosomal recessive inheritance
HP:0000023	Inguinal hernia
HP:0000160	Narrow mouth
HP:0000175	Cleft palate
HP:0000238	Hydrocephalus
HP:0000248	Brachycephaly
HP:0000274	Small face
HP:0000308	Microretrognathia
HP:0000316	Hypertelorism
HP:0000337	Broad forehead

Displaying 1 entry
Disease ID	Disease Name
OMIM:245600	Larsen-like syndrome, B3GAT3 type

PubChem Disease

MedGen Diseases

Larsen-like syndrome, B3GAT3 type

OMIM Phenotypes

MULTIPLE JOINT DISLOCATIONS, SHORT STATURE, AND CRANIOFACIAL DYSMORPHISM WITH OR WITHOUT CONGENITAL HEART DEFECTS; JDSCD

KEGG Diseases

Multiple joint dislocations, short stature, craniofacial dysmorphism, and congenital heart defects

Gene-Disease-Co-Occurrences-in-Literature (PubChem)

Ortholog

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Species	Gene ID	Alliance of Genome Resources	Orthologous MAtrix
Xenopus tropicalis	26229	Xenbase:XB-GENE-1010243
Drosophila melanogaster	27087	FB:FBgn0066114
Caenorhabditis elegans	34282	WB:WBGene00008479
Caenorhabditis elegans	39262	WB:WBGene00008479
Drosophila melanogaster	64544	FB:FBgn0066114
Mus musculus	72727	Xenbase:XB-GENE-1010243	MOUSE25901
Drosophila melanogaster	76898	FB:FBgn0066114
Drosophila melanogaster	117108	FB:FBgn0066114
Drosophila melanogaster	135152	FB:FBgn0066114
Xenopus tropicalis	174517	Xenbase:XB-GENE-1010243

GlyTouCan

GlyComb

GlycoPOST

UniCarb-DR

LM-GlycoRepo

All Resources

Other Information

Tools

Search Tools

Guidelines

Summary

External Links

Annotation

OrthoDB (Group)

GlycoGene Database (GGDB)

KEGG BRITE Database

PubChem Disease

International Collaboration

Acknowledgements